Iduronidase
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. It catalyses the hydrolysis of unsulfated α-L-iduronosidic linkages in dermatan sulfate.
| 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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| 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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| Formula | C3567H5645N921O1261P4S12 |
| Molar mass | 82117.20 g·mol−1 |
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.
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