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Th1131 details

Primary information
ID 1608
ThPP ID Th1131
Therapeutic Peptide/Protein Name Taliglucerase alfa
Sequence EFARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRR view full sequnce in fasta
Functional Classification Ia
Molecular Weight 56637.9397
Chemical Formula C2580H3918N680O727S17
Isoelectric Point 10.54
Hydrophobicity N.A.
Melting Point (℃) N.A.
Half Life N.A.
Description Recombinant human glucocerebrosidase (a lysosomal enzyme) . Elelyso used in patients with type 1 Gaucher's disease
Indication/Disease For the treatment of adult Type 1 Gaucher disease.
Pharmacodynamics Patient's with Type 1 Gaucher disease have a long-term deficiency in the enzyme, glucocerebrosidase. Taliglucerase alfa is a modified form of glucocerebrosidase and is provided to counter this enzyme deficiency, resulting in smaller liver and spleen size, and improved thrombocytopenia and anemia.
Mechanism of Action Taliglucerase alfa is different from human glucocerebrosidase by two amino acids at the N terminal and up to 7 amino acids at the C terminal. This recombinant enzyme allows the hydrolysis reaction of glucocerebroside to glucose and ceramide that naturally occurs in healthy individuals.
Toxicity The most common toxic reaction seen was infusion reactions such as urticaria, arthralgia, headache, and chest pain due to IV administration.
Metabolism Metabolism was not determined.
Absorption Taliglucerase alfa is administered IV so absorption is 100%.
Volume of Distribution The steady state volume of distribution is between 7.30 to 11.7 L.
Clearance The systemic clearance was approximately 30 L/hr and 20 L/hr for 30 and 60 units/kg, respectively.
Categories Enzymes
Patents Number N.A.
Date of Issue N.A.
Date of Expiry N.A.
Drug Interaction N.A.
Target Glucocerebroside
Information of corresponding available drug in the market
Brand Name Elelyso
Company Pfizer
Brand Discription Taliglucerase alfa, a hydrolytic lysosomal glucocerebroside-specific enzyme for Intravenous infusion, is a recombinant active form of the lysosomal enzyme, Î²-glucocerebrosidase, which is expressed in genetically modified carrot plant root cells cultured in a disposable bioreactor system (ProCellEx). Î²-Glucocerebrosidase (Î²-D-glucosyl-Nacylsphingosine glucohydrolase, E.C. 3.2.1.45) is a lysosomal glycoprotein enzyme that catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide. ELELYSO is produced by recombinant DNA technology using plant cell culture (carrot). Purified taliglucerase alfa is a monomeric glycoprotein containing 4 N-linked glycosylation sites (Mr = 60,800). Taliglucerase alfa differs from native human glucocerebrosidase by two amino acids at the N terminal and up to 7 amino acids at the C terminal. Taliglucerase alfa is a glycosylated protein with oligosaccharide chains at the glycosylation sites having terminal mannose sugars. These mannose-terminated oligosaccharide chains of taliglucerase alfa are specifically recognized by endocytic carbohydrate receptors on macrophages, the cells that accumulate lipid in Gaucher disease.
Prescribed for long-term enzyme replacement therapy (ERT) for adult and pediatric patients with a confirmed diagnosis of Type 1 Gaucher disease.
Chemical Name N.A.
Formulation The quantitative composition of each 200 unit vial is D-mannitol (206.7 mg), polysorbate 80 (0.56 mg), sodium citrate (30.4 mg), and taliglucerase alfa (212 units). Citric acid may be added to adjust the pH at the time of manufacture.
Physcial Appearance ELELYSO is supplied as a sterile, non-pyrogenic, lyophilized powder.
Route of Administration Intravenous infusion
Recommended Dosage 60 units per kg of body weight
Contraindication N.A.
Side Effects pruritus, flushing, headache, arthralgia, pain in extremity, abdominal pain, vomiting, fatigue, back pain, dizziness, nausea, and rash.
Useful Link http://www.rxlist.com/elelyso-drug.htm http://www.elelyso.com/ http://www.drugs.com/elelyso.html
PubMed ID 23430813, 25453586, 24950666, 24630271, 23199589, 21900191
3-D Structure Th1131 (View) or (Download)

OSDDlinux

Raghava's Group

IMTECH

CSIR

CRDD
CPPSITE 2.0

Primary information
ID	1608
ThPP ID	Th1131
Therapeutic Peptide/Protein Name	Taliglucerase alfa
Sequence	EFARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRR view full sequnce in fasta
Functional Classification	Ia
Molecular Weight	56637.9397
Chemical Formula	C2580H3918N680O727S17
Isoelectric Point	10.54
Hydrophobicity	N.A.
Melting Point (℃)	N.A.
Half Life	N.A.
Description	Recombinant human glucocerebrosidase (a lysosomal enzyme) . Elelyso used in patients with type 1 Gaucher's disease
Indication/Disease	For the treatment of adult Type 1 Gaucher disease.
Pharmacodynamics	Patient's with Type 1 Gaucher disease have a long-term deficiency in the enzyme, glucocerebrosidase. Taliglucerase alfa is a modified form of glucocerebrosidase and is provided to counter this enzyme deficiency, resulting in smaller liver and spleen size, and improved thrombocytopenia and anemia.
Mechanism of Action	Taliglucerase alfa is different from human glucocerebrosidase by two amino acids at the N terminal and up to 7 amino acids at the C terminal. This recombinant enzyme allows the hydrolysis reaction of glucocerebroside to glucose and ceramide that naturally occurs in healthy individuals.
Toxicity	The most common toxic reaction seen was infusion reactions such as urticaria, arthralgia, headache, and chest pain due to IV administration.
Metabolism	Metabolism was not determined.
Absorption	Taliglucerase alfa is administered IV so absorption is 100%.
Volume of Distribution	The steady state volume of distribution is between 7.30 to 11.7 L.
Clearance	The systemic clearance was approximately 30 L/hr and 20 L/hr for 30 and 60 units/kg, respectively.
Categories	Enzymes
Patents Number	N.A.
Date of Issue	N.A.
Date of Expiry	N.A.
Drug Interaction	N.A.
Target	Glucocerebroside
Information of corresponding available drug in the market
Brand Name	Elelyso
Company	Pfizer
Brand Discription	Taliglucerase alfa, a hydrolytic lysosomal glucocerebroside-specific enzyme for Intravenous infusion, is a recombinant active form of the lysosomal enzyme, Î²-glucocerebrosidase, which is expressed in genetically modified carrot plant root cells cultured in a disposable bioreactor system (ProCellEx). Î²-Glucocerebrosidase (Î²-D-glucosyl-Nacylsphingosine glucohydrolase, E.C. 3.2.1.45) is a lysosomal glycoprotein enzyme that catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide. ELELYSO is produced by recombinant DNA technology using plant cell culture (carrot). Purified taliglucerase alfa is a monomeric glycoprotein containing 4 N-linked glycosylation sites (Mr = 60,800). Taliglucerase alfa differs from native human glucocerebrosidase by two amino acids at the N terminal and up to 7 amino acids at the C terminal. Taliglucerase alfa is a glycosylated protein with oligosaccharide chains at the glycosylation sites having terminal mannose sugars. These mannose-terminated oligosaccharide chains of taliglucerase alfa are specifically recognized by endocytic carbohydrate receptors on macrophages, the cells that accumulate lipid in Gaucher disease.
Prescribed for	long-term enzyme replacement therapy (ERT) for adult and pediatric patients with a confirmed diagnosis of Type 1 Gaucher disease.
Chemical Name	N.A.
Formulation	The quantitative composition of each 200 unit vial is D-mannitol (206.7 mg), polysorbate 80 (0.56 mg), sodium citrate (30.4 mg), and taliglucerase alfa (212 units). Citric acid may be added to adjust the pH at the time of manufacture.
Physcial Appearance	ELELYSO is supplied as a sterile, non-pyrogenic, lyophilized powder.
Route of Administration	Intravenous infusion
Recommended Dosage	60 units per kg of body weight
Contraindication	N.A.
Side Effects	pruritus, flushing, headache, arthralgia, pain in extremity, abdominal pain, vomiting, fatigue, back pain, dizziness, nausea, and rash.
Useful Link	http://www.rxlist.com/elelyso-drug.htm http://www.elelyso.com/ http://www.drugs.com/elelyso.html
PubMed ID	23430813, 25453586, 24950666, 24630271, 23199589, 21900191
3-D Structure	Th1131 (View) or (Download)

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