About: Alglucosidase alfa

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An Entity of Type : http://linked.opendata.cz/ontology/drugbank/Drug, within Data Space : linked.opendata.cz associated with source document(s)

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rdf:type	http://linked.opendata.cz/ontology/drugbank/Drug
http://linked.open...gbank/description	Aglucosidase alfa consists of the human enzyme acid alpha-glucosidase (GAA) which is essential for the degradation of glygogen to glucose in lysosomes. It is encoded by the most predominant of nine observed haplotypes of this gene. Aglucosidase alfa is produced by recombinant DNA technology in a Chinese hamster ovary cell line. Alglucosidase alfa degrades glycogen by catalyzing the hydrolysis of a-1,4- and a-1,6- glycosidic linkages of lysosomal glycogen. Structurally, Alglucosidase alfa is a glycoprotein with a calculated mass of 98,008 daltons for the 883 residue mature polypeptide chain, and a total mass of approximately 109,000 daltons, including carbohydrates. It is used for the treatment of Pompe disease (GAA deficiency) in infants and pediatric patients. (en)
http://linked.open...y/drugbank/dosage	http://linked.opendata.cz/resource/drugbank/dosage/271B61B7-363D-11E5-9242-09173F13E4C5 http://linked.opendata.cz/resource/drugbank/dosage/271B61B8-363D-11E5-9242-09173F13E4C5 http://linked.opendata.cz/resource/drugbank/dosage/271B61B9-363D-11E5-9242-09173F13E4C5
http://linked.open...generalReferences	# Kishnani PS, Corzo D, Nicolino M, Byrne B, Mandel H, Hwu WL, Leslie N, Levine J, Spencer C, McDonald M, Li J, Dumontier J, Halberthal M, Chien YH, Hopkin R, Vijayaraghavan S, Gruskin D, Bartholomew D, van der Ploeg A, Clancy JP, Parini R, Morin G, Beck M, De la Gastine GS, Jokic M, Thurberg B, Richards S, Bali D, Davison M, Worden MA, Chen YT, Wraith JE: Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology. 2007 Jan 9;68(2):99-109. Epub 2006 Dec 6. "Pubmed":http://www.ncbi.nlm.nih.gov/pubmed/17151339 (en)
http://linked.open...gy/drugbank/group	approved (en)
http://linked.open...drugbank/halfLife	2.3 ± 0.4 hours. (en)
http://linked.open...ugbank/indication	For the treatment of Pompe disease (GAA deficiency) in infants and pediatric patients. (en)
sameAs	Alglucosidase alfa
Title	Alglucosidase alfa (en)
adms:identifier	http://linked.opendata.cz/resource/drugbank/drug/DB01272/identifier/drugbank/DB01272 http://linked.opendata.cz/resource/drugbank/drug/DB01272/identifier/kegg-drug/D03207 http://linked.opendata.cz/resource/drugbank/drug/DB01272/identifier/national-drug-code-directory/58468-0150-1 http://linked.opendata.cz/resource/drugbank/drug/DB01272/identifier/pharmgkb/PA164754753 http://linked.opendata.cz/resource/drugbank/drug/DB01272/identifier/wikipedia/Alglucosidase_alfa
http://linked.open...mechanismOfAction	Alglucosidase alfa is designed to act as an exogenous source of GAA, acting to correct GAA deficiency that is the hallmark of Pompe disease. Alglucosidase alfa binds to mannose-6-phosphate receptors on the cell surface via carbohydrate groups on the GAA molecule, after which it is internalized and transported into lysosomes, where it undergoes proteolytic cleavage that results in increased enzymatic activity. It then exerts enzymatic activity in cleaving glycogen. Specifically, it hydrolyses alpha-1,4-glucose bonds. (en)
http://linked.open...drugbank/packager	Genzyme Inc.
http://linked.open...y/drugbank/patent	http://linked.opendata.cz/resource/drugbank/patent/2416492
http://linked.open...outeOfElimination	Via kidney and liver (en)
http://linked.open.../drugbank/synonym	Acid maltase (en) Acid-alpha glucosidase (en) Aglucosidase alfa (en) Aglucosidase alpha (en) Alpha-glucosidase (en) Lysosomal Alpha-Glucosidase (en) alpha-1,4-glucosidase (en)
http://linked.open...drugbank/toxicity	There have been no reports of overdose with alglucosidase alfa. (en)
http://linked.open...umeOfDistribution	* 96 ± 16 mL/kg [20 mg/kg dose] * 119 ± 28 mL/kg [40 mg/kg dose] (en)
http://linked.open.../drug/hasAHFSCode	http://linked.opendata.cz/resource/AHFS/44-00-00
foaf:page	http://www.drugs.com/cdi/alglucosidase-alfa.html http://www.rxlist.com/cgi/generic/myozyme.htm
http://linked.open...Molecular-Formula	http://linked.opendata.cz/resource/drugbank/property/271B61BB-363D-11E5-9242-09173F13E4C5
http://linked.open.../Molecular-Weight	http://linked.opendata.cz/resource/drugbank/property/271B61BA-363D-11E5-9242-09173F13E4C5
http://linked.open...l/drug/hasATCCode	Alglucosidase alfa
http://linked.open.../affectedOrganism	Humans and other mammals (en)
http://linked.open...casRegistryNumber	420784-05-0 (en)
http://linked.open...drugbank/category	(en)
http://linked.open...rugbank/clearance	* 25+/- 4 mL/hr/kg [4-hour IV infusion of 20 mg/kg] (en)
http://linked.open...gbank/containedIn	Myozyme 50 mg vial

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