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| Primary information |
|---|
| ID | 12789 |
| Therapeutic ID | Th1355 |
| Protein Name | Human C1-esterase inhibitor |
| Sequence | >Th1355_Human_C1-esterase_inhibitor
MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA
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| 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 |