Iduronidase
Mammalian protein found in ''Homo sapiens'' / From Wikipedia, the free encyclopedia
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Iduronidase (EC 3.2.1.76, L-iduronidase, α-L-iduronidase, laronidase), sold as Aldurazyme, is an enzyme with the systematic name glycosaminoglycan α-L-iduronohydrolase.[3][4][5] It catalyses the hydrolysis of unsulfated α-L-iduronosidic linkages in dermatan sulfate.[6]
Quick Facts Identifiers, Symbol ...
iduronidase, α-L- | |||||||
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Identifiers | |||||||
Symbol | IDUA | ||||||
NCBI gene | 3425 | ||||||
HGNC | 5391 | ||||||
OMIM | 252800 | ||||||
RefSeq | NM_000203 | ||||||
UniProt | P35475 | ||||||
Other data | |||||||
EC number | 3.2.1.76 | ||||||
Locus | Chr. 4 p16.3 | ||||||
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Quick Facts Clinical data, Trade names ...
Clinical data | |
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Trade names | Aldurazyme |
Other names | alpha-L-Idosiduronase, Laronidase (genetical recombination) (JAN) (JAN JP) |
AHFS/Drugs.com | Monograph |
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Routes of administration | Intravenous (IV) |
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Chemical and physical data | |
Formula | C3567H5645N921O1261P4S12 |
Molar mass | 82117.20 g·mol−1 |
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It is a glycoprotein enzyme found in the lysosomes of cells. It is involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. The enzyme acts by hydrolyzing the terminal α-L-iduronic acid residues of these molecules, degrading them. The protein is reported as having a mass of approximately 83 kDa.[6]