alpha-L-iduronidase (laronidase, Aldurazyme, IDUA)

Function

• hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate

Structure

• monomer (probable)
• belongs to the glycosyl hydrolase 39 family

Compartment

lysosome

Expression

ubiquitous

Pathology

• defects in IDUA are the cause of
  • Hurler-Scheie syndrome

Pharmacology

• see Aldurazyme

Laboratory

• IDUA gene mutation
• alpha-L-iduronidase in fibroblasts
• alpha-L-iduronidase in leukocytes

More general terms

• iduronidase
• metabolic agent (metabolic modifier)

More specific terms

• Aldurazyme (alpha-L-iduronidase)

Database

• OMIM: https://mirror.omim.org/entry/252800
• OMIM: https://mirror.omim.org/entry/607014
• OMIM: https://mirror.omim.org/entry/607015
• OMIM: https://mirror.omim.org/entry/607016
• UniProt: http://www.uniprot.org/uniprot/P35475.html