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Namespace Prefixes

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Statements

Subject Item
n2:DB08876
rdf:type
n3:Drug
n3:description
Taliglucerase alfa is the recombinant active form of the human lysosomal enzyme, β-glucocerebrosidase. It was approved in 2012 and is marketed under the name Elelyso for use in patients with type 1 Gaucher's disease.
n3:dosage
n17:271B59FE-363D-11E5-9242-09173F13E4C5 n17:271B59FF-363D-11E5-9242-09173F13E4C5
n3:generalReferences
# Zimran A, Brill-Almon E, Chertkoff R, Petakov M, Blanco-Favela F, Munoz ET, Solorio-Meza SE, Amato D, Duran G, Giona F, Heitner R, Rosenbaum H, Giraldo P, Mehta A, Park G, Phillips M, Elstein D, Altarescu G, Szleifer M, Hashmueli S, Aviezer D: Pivotal trial with plant cell-expressed recombinant glucocerebrosidase, taliglucerase alfa, a novel enzyme replacement therapy for Gaucher disease. Blood. 2011 Nov 24;118(22):5767-73. doi: 10.1182/blood-2011-07-366955. Epub 2011 Sep 6. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/21900191 # Aviezer D, Brill-Almon E, Shaaltiel Y, Hashmueli S, Bartfeld D, Mizrachi S, Liberman Y, Freeman A, Zimran A, Galun E: A plant-derived recombinant human glucocerebrosidase enzyme--a preclinical and phase I investigation. PLoS One. 2009;4(3):e4792. doi: 10.1371/journal.pone.0004792. Epub 2009 Mar 11. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/19277123
n3:group
approved
n3:halfLife
The half life is between 18.9 to 28.7 min.
n3:indication
For the treatment of adult Type 1 Gaucher disease.
owl:sameAs
n9:DB08876
dcterms:title
Taliglucerase Alfa
adms:identifier
n14:DB08876 n15:D09675 n16:Taliglucerase_alfa
n3:mechanismOfAction
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.
n3:routeOfElimination
Route of elimination was not determined.
n3:synonym
prGCD
n3:toxicity
The most common toxic reaction seen was infusion reactions such as urticaria, arthralgia, headache, and chest pain due to IV administration.
n3:volumeOfDistribution
The steady state volume of distribution is between 7.30 to 11.7 L.
n3:foodInteraction
No food effects found.
n3:proteinBinding
Plasma protein binding was not quantified.
n3:synthesisReference
Shaaltiel Y, Bartfeld D, Hashmueli S, Baum G, Brill-Almon E, Galili G, Dym O, Boldin-Adamsky SA, Silman I, Sussman JL, Futerman AH, Aviezer D: Production of glucocerebrosidase with terminal mannose glycans for enzyme replacement therapy of Gaucher's disease using a plant cell system. Plant Biotechnol J. 2007 Sep;5(5):579-90. Epub 2007 May 24.
foaf:page
n7:taliglucerase-alfa.html n10:elelyso-drug.htm
n3:Molecular-Formula
n4:271B5A02-363D-11E5-9242-09173F13E4C5
n3:Molecular-Weight
n4:271B5A01-363D-11E5-9242-09173F13E4C5
n3:Water-Solubility
n4:271B5A00-363D-11E5-9242-09173F13E4C5
n11:hasATCCode
n12:A16AB11
n3:absorption
Taliglucerase alfa is administered IV so absorption is 100%.
n3:affectedOrganism
Humans and other mammals
n3:casRegistryNumber
37228-64-1
n3:category
n3:clearance
The systemic clearance was approximately 30 L/hr and 20 L/hr for 30 and 60 units/kg, respectively.