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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
Laboratory:
- IDUA gene mutation
- alpha-L-iduronidase in fibroblasts
- alpha-L-iduronidase in leukocytes
Pharmacology:
- see Aldurazyme
Related
IDUA gene mutation
Specific
Aldurazyme (alpha-L-iduronidase)
Alpha Iduronidase Parenteral
General
iduronidase
metabolic agent (metabolic modifier)
Properties
SIZE: entity length = 653 aa
MW = 73 kD
COMPARTMENT: lysosome
MOTIF: signal sequence {1-27}
N-glycosylation site {N110}
glutamate residue {E182}
N-glycosylation site {N190}
glutamate residue {E299}
N-glycosylation site {N336}
N-glycosylation site {N372}
N-glycosylation site {N415}
N-glycosylation site {N451}
Database Correlations
OMIM correlations
MORBIDMAP 252800
UniProt P35475
Pfam PF01229
ENZYME 3.2.1.76
References
- UniProt :accession P35475
- Prescriber's Letter 11(2):suppl 2004
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=IDUA