Detailed description page of ThPDB2
| This page displays user query in tabular form. |
Th1355 details |
| Primary information | |
|---|---|
| ID | 12778 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Amino Acids, Peptides, and Proteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Berinert |
| Company | CSL Behring GmbH |
| Brand Description | CSL Behring GmbH |
| Prescribed For | Intravenous |
| Chemical Name | 500 [iU]/10mL |
| Formulation | BERINERT is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor preparations. |
| Physical Appearance | HAE attack headache abdominal or stomach pain nausea muscle spasms general pain diarrhea vomiting changes in taste cold symptoms (stuffy nose, sneezing, soe throat) itching rash, and allergic reactions |
| Route of Administration | Berinert is a man-made form of a protein that occurs naturally in the blood stream and helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein. Hereditary angioedema can cause attacks of swelling and symptoms such as stomach problems... |
| Recommended Dosage | BERINERT is a plasma-derived concentrate of C1 Esterase Inhibitor (Human) indicated for the treatment of acute abdominal, facial, or laryngeal hereditary angioedema (HAE) attacks in adult and pediatric patients. |
| Contraindication | NA |
| Side Effects | Tell your healthcare provider about any side effect that bothers you or that does not go away. You can also report side effects to the FDA at 1-800-FDA-1088. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12779 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Blood and Blood Forming Organs |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Berinert 1500 |
| Company | Csl Behring |
| Brand Description | Csl Behring |
| Prescribed For | Intravenous |
| Chemical Name | 1500 unit / vial |
| Formulation | BERINERT is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor preparations. |
| Physical Appearance | The most serious adverse reaction reported in subjects enrolled in clinical studies who received Berinert was an increase in the severity of pain associated with HAE. The most common adverse reaction reported in greater than 4% of the subjects and greater than placebo among subjects who received Berinert in the placebo-controlled clinical trial was dysgeusia. |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12780 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Blood Proteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Berinert 500 |
| Company | Csl Behring |
| Brand Description | Csl Behring |
| Prescribed For | Intravenous |
| Chemical Name | 500 unit / vial |
| Formulation | BERINERT is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor preparations. |
| Physical Appearance | The most serious adverse reaction reported in subjects enrolled in clinical studies who received Berinert was an increase in the severity of pain associated with HAE. The most common adverse reaction reported in greater than 4% of the subjects and greater than placebo among subjects who received Berinert in the placebo-controlled clinical trial was dysgeusia. |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12781 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | C1 inhibitors |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Cinryze |
| Company | Viropharma Biologics Llc |
| Brand Description | Viropharma Biologics Llc |
| Prescribed For | Intravenous |
| Chemical Name | 500 [iU]/5mL |
| Formulation | CINRYZE is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis to the product. |
| Physical Appearance | headache nausea vomiting stomach pain changes in sense of taste cold symptoms (stuffy nose, sneezing, sore throat), or mild itching or rash |
| Route of Administration | Cinryze is a man-made form of a protein that occurs naturally in the blood stream and helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein. Hereditary angioedema can cause attacks of swelling and symptoms such as stomach problems... |
| Recommended Dosage | CINRYZE is a C1 esterase inhibitor indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE). |
| Contraindication | NA |
| Side Effects | CINRYZE, when reconstituted with 5 mL of Sterile Water for Injection, USP contains the following excipients: 4.1 mg/mL sodium chloride, 21 mg/mL sucrose, 2.6 mg/mL trisodium citrate, 2.0 mg/mL LValine, 1.2 mg/mL L-Alanine, and 4.5 mg/mL L-Threonine. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12782 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Carbohydrates |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Cinryze |
| Company | Takeda |
| Brand Description | Takeda |
| Prescribed For | Intravenous |
| Chemical Name | 500 unit / vial |
| Formulation | CINRYZE is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis to the product. |
| Physical Appearance | headache nausea vomiting stomach pain changes in sense of taste cold symptoms (stuffy nose, sneezing, sore throat), or mild itching or rash |
| Route of Administration | Cinryze is a man-made form of a protein that occurs naturally in the blood stream and helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein. Hereditary angioedema can cause attacks of swelling and symptoms such as stomach problems... |
| Recommended Dosage | CINRYZE is a C1 esterase inhibitor indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE). |
| Contraindication | NA |
| Side Effects | CINRYZE, when reconstituted with 5 mL of Sterile Water for Injection, USP contains the following excipients: 4.1 mg/mL sodium chloride, 21 mg/mL sucrose, 2.6 mg/mL trisodium citrate, 2.0 mg/mL LValine, 1.2 mg/mL L-Alanine, and 4.5 mg/mL L-Threonine. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12783 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Complement C1 Inactivator Proteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Cinryze |
| Company | Shire Services Bvba |
| Brand Description | Shire Services Bvba |
| Prescribed For | Intravenous |
| Chemical Name | 500 U |
| Formulation | CINRYZE is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis to the product. |
| Physical Appearance | headache nausea vomiting stomach pain changes in sense of taste cold symptoms (stuffy nose, sneezing, sore throat), or mild itching or rash |
| Route of Administration | Cinryze is a man-made form of a protein that occurs naturally in the blood stream and helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein. Hereditary angioedema can cause attacks of swelling and symptoms such as stomach problems... |
| Recommended Dosage | CINRYZE is a C1 esterase inhibitor indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE). |
| Contraindication | NA |
| Side Effects | CINRYZE, when reconstituted with 5 mL of Sterile Water for Injection, USP contains the following excipients: 4.1 mg/mL sodium chloride, 21 mg/mL sucrose, 2.6 mg/mL trisodium citrate, 2.0 mg/mL LValine, 1.2 mg/mL L-Alanine, and 4.5 mg/mL L-Threonine. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12784 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Complement Inactivating Agents |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Cinryze |
| Company | Viropharma Biologics Llc |
| Brand Description | Viropharma Biologics Llc |
| Prescribed For | Intravenous |
| Chemical Name | 500 [iU]/5mL |
| Formulation | CINRYZE is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis to the product. |
| Physical Appearance | headache nausea vomiting stomach pain changes in sense of taste cold symptoms (stuffy nose, sneezing, sore throat), or mild itching or rash |
| Route of Administration | Cinryze is a man-made form of a protein that occurs naturally in the blood stream and helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein. Hereditary angioedema can cause attacks of swelling and symptoms such as stomach problems... |
| Recommended Dosage | CINRYZE is a C1 esterase inhibitor indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE). |
| Contraindication | NA |
| Side Effects | CINRYZE, when reconstituted with 5 mL of Sterile Water for Injection, USP contains the following excipients: 4.1 mg/mL sodium chloride, 21 mg/mL sucrose, 2.6 mg/mL trisodium citrate, 2.0 mg/mL LValine, 1.2 mg/mL L-Alanine, and 4.5 mg/mL L-Threonine. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12785 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Complement Inactivator Proteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Haegarda |
| Company | Csl Behring |
| Brand Description | Csl Behring |
| Prescribed For | Subcutaneous |
| Chemical Name | 2000 unit / vial |
| Formulation | HAEGARDA is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1-INH preparations or its excipients [see DESCRIPTION]. |
| Physical Appearance | injection site reactions (bruising, cold feeing, discharge, redness, swelling, itching, pain, rash, scarring, and warmth), hypersensitivity (itching, rash, hives), runny or stuffy nose, and dizziness. |
| Route of Administration | Haegarda is a man-made form of a protein in blood that helps control swelling in the body. People with a condition called Hereditary Angioedema (HAE) do not have enough of this protein. Hereditary Angioedema can cause attacks of swelling and symptoms such as stomach problems or trouble breathing. Haegarda... |
| Recommended Dosage | Haegarda is a prescription medicine used as prophylaxis to Hereditary Angioedema. Haegarda may be used alone or with other medications. |
| Contraindication | NA |
| Side Effects | HAEGARDA is a human plasma-derived, purified, pasteurized, lyophilized concentrate of C1-INH to be reconstituted for S.C. administration. HAEGARDA is prepared from large pools of human plasma from U.S. donors. The potency of C1-INH is expressed in International Units (IU), which is related to the current WHO Standard for C1-INH products. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12786 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Complement System Proteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | Haegarda |
| Company | Csl Behring |
| Brand Description | Csl Behring |
| Prescribed For | Subcutaneous |
| Chemical Name | 3000 unit / vial |
| Formulation | HAEGARDA is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1-INH preparations or its excipients [see DESCRIPTION]. |
| Physical Appearance | injection site reactions (bruising, cold feeing, discharge, redness, swelling, itching, pain, rash, scarring, and warmth), hypersensitivity (itching, rash, hives), runny or stuffy nose, and dizziness. |
| Route of Administration | Haegarda is a man-made form of a protein in blood that helps control swelling in the body. People with a condition called Hereditary Angioedema (HAE) do not have enough of this protein. Hereditary Angioedema can cause attacks of swelling and symptoms such as stomach problems or trouble breathing. Haegarda... |
| Recommended Dosage | Haegarda is a prescription medicine used as prophylaxis to Hereditary Angioedema. Haegarda may be used alone or with other medications. |
| Contraindication | NA |
| Side Effects | HAEGARDA is a human plasma-derived, purified, pasteurized, lyophilized concentrate of C1-INH to be reconstituted for S.C. administration. HAEGARDA is prepared from large pools of human plasma from U.S. donors. The potency of C1-INH is expressed in International Units (IU), which is related to the current WHO Standard for C1-INH products. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12787 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Decreased Vascular Permeability |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | HAEGARDA C1 Esterase Inhibitor Subcutaneous (Human) |
| Company | CSL Behring GmbH |
| Brand Description | CSL Behring GmbH |
| Prescribed For | Subcutaneous |
| Chemical Name | 2000 [iU]/4mL |
| Formulation | HAEGARDA is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1-INH preparations or its excipients [see DESCRIPTION]. |
| Physical Appearance | Cough difficulty swallowing dizziness fast heartbeat hives, itching, or skin rash pains in the chest, groin, or legs, especially calves of the legs severe headaches of sudden onset shortness of breath sudden loss of coordination sudden onset of shortness of breath for no apparent reason sudden onset of slurred speech sudden vision changes tightness in the chest unusual tiredness or weakness |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12788 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Drugs Used in Hereditary Angioedema |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | HAEGARDA C1 Esterase Inhibitor Subcutaneous (Human) |
| Company | CSL Behring GmbH |
| Brand Description | CSL Behring GmbH |
| Prescribed For | Subcutaneous |
| Chemical Name | 3000 [iU]/6mL |
| Formulation | HAEGARDA is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1-INH preparations or its excipients [see DESCRIPTION]. |
| Physical Appearance | Cough difficulty swallowing dizziness fast heartbeat hives, itching, or skin rash pains in the chest, groin, or legs, especially calves of the legs severe headaches of sudden onset shortness of breath sudden loss of coordination sudden onset of shortness of breath for no apparent reason sudden onset of slurred speech sudden vision changes tightness in the chest unusual tiredness or weakness |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12789 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Enzyme Inhibitors |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12790 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Glycoconjugates |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12791 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Glycoproteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12792 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Immunologic Factors |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12793 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Immunoproteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12794 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Kallikrein Inhibitors |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12795 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Peptides |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12796 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Protease Inhibitors |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12797 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Proteins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12798 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Serine Protease Inhibitors |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |
| Primary information | |
|---|---|
| ID | 12799 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA |
| Molecular Weight | 68000 |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The half life of a 50 IU/kg dose is 2.4 ± 0.6 h, while the half life of a 100 IU/kg dose is 2.7 ± 0.3 h.[L16591] Subcutaneous administration produces a half life of 199.6 hours.[A19661] |
| Description | C1 Esterase Inhibitor (Human) is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma.[L16586,L16591,L16606] The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways.[L16586,L16591,L16606] This drug is indicated for prophylaxis and treatment of Hereditary Angioedema (HAE), a human genetic disorder caused by a shortage of C1 inhibitor activity that results in an overreaction of the immune system.[L16586,L16591,L16606] The disease is characterized by acute attacks of painful and in some cases fatal swelling of several soft tissues (edema), which may last up to five days when untreated.[L16586,L16591,L16606] |
| Indication/Disease | C1 esterase inhibitors are indicated for the prophylaxis and treatment of acute attacks of hereditary angioedema in patients at least 6 years old.[L16586,L16591,L16596] |
| Pharmacodynamics | The C1 esterase inhibitor treats and prevents attacks of hereditary angioedema.[L16591] It has a long duration of action as it is given every 3-4 days prophylactically.[L16586] Patients should be counselled regarding the risk of hypersensitivity reactions as well as arterial and venous thromboemboli.[L16586,L16591] |
| Mechanism of Action | The C1 esterase inhibitor binds to proteins such as C1s, kallikrein, factor XIIa, and XIa and irreversibly inactivates them.[L16586,L16591] Patients with hereditary angioedema have low levels of C1 esterase inhibitors.[L16586,L16591] By providing patients with C1 esterase inhibitors, this may prevent contact system activation, which prevents increases in vascular permeability.[L16586,L16591] Manifestations of hereditary angioedema may be prevented by preventing increases in vascular permeability.[L16586,L16591] |
| Toxicity | Data regarding overdoses in humans are not readily available.[L16591] A study in monkeys showed doses above the recommended human dose resulted in temporarily increased liver enzymes such as AST and ALP.[L16591] |
| Metabolism | Protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A 50 IU/kg dose reaches a Cmax of 1.2 ± 0.2 IU/mL, with a Tmax of 0.31 ± 0.10 hours, and an AUC of 3.3 ± 1.0 IU |
| The volume of distribution at steady state of a 50 IU/kg dose is 3.0 ± 0.9 L.[L16591] | |
| Clearance | The clearance of a 50 IU/kg dose is 1207 ± 414 mL/h, while the clearance of a 100 IU/kg dose is 781 ± 147 mL/h.[L16591] |
| Categories | Serpins |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Complement C1r subcomponent,Complement C1s subcomponent,Plasma kallikrein,Coagulation factor XII,Prothrombin,Coagulation factor XI,Tissue-type plasminogen activator |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |