Iduronidase

iduronidase, α-L-
Identifiers
SymbolIDUA
NCBI gene3425
HGNC5391
OMIM252800
RefSeqNM_000203
UniProtP35475
Other data
EC number3.2.1.76
LocusChr. 4 p16.3
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StructuresSwiss-model
DomainsInterPro

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.

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.