Protein Name alpha-L-iduronidase
Organism Homo sapiens
Gene ID 3425
Gene Symbol

IDUA

UniProt P35475 (IDUA_HUMAN)
Relationships Total Number of functionally related compound(s) : 149
Total Number of Articles : 169
Description

alpha-L-iduronidase

Gene Summary

This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). [provided by RefSeq, Jul 2008]

synonyms
  • alpha-L-iduronidase
  • iduronidase alpha-L-
  • mucopolysaccharidosis type I
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Properties