Iduronidase
| iduronidase, α-L- | |||||||
|---|---|---|---|---|---|---|---|
| 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 | ||||||
| |||||||
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.[1][2][3] It catalyses the hydrolysis of unsulfated α-L-iduronosidic linkages in dermatan sulfate.[4]
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.[4]
- ^ Matalon R, Cifonelli JA, Dorfman A (January 1971). "L-Iduronidase in cultured human fibroblasts and liver". Biochemical and Biophysical Research Communications. 42 (2): 340–5. Bibcode:1971BBRC...42..340M. doi:10.1016/0006-291x(71)90108-2. PMID 4993544.
- ^ Rome LH, Garvin AJ, Neufeld EF (August 1978). "Human kidney α-L-iduronidase: purification and characterization". Archives of Biochemistry and Biophysics. 189 (2): 344–53. doi:10.1016/0003-9861(78)90221-7. PMID 30407.
- ^ Srivastava RM, Hudson N, Seymour FR, Weissman B (1978). "Preparation of (aryl α-L-idopyranosid)uronic acids". Carbohydr. Res. 60 (2): 315–326. doi:10.1016/s0008-6215(78)80038-x.
- ^ a b Aldurazyme (Laronidase). BioMarin Pharmaceuticals Inc. FDA website. Retrieved 6 December 2015.