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iduronate-2-sulfatase (IDS, alpha-L-iduronate sulfate sulfatase)
Function:
- required for the lysosomal degradation of heparan sulfate & dermatan sulfate
- hydrolysis of the 2-sulfate groups of L-iduronate 2-sulfate units of dermatan sulfate, heparan sulfate & heparin
Cofactor: binds 1 Ca+2 per subunit (putative)
Structure: belongs to the sulfatase family
Compartment: lysosome
Alternative splicing: named isoforms=2
Expression: liver, kidney, lung, & placenta
Pathology:
- defects are associated with Hunter syndrome (mucopolysaccharidosis 2)
Laboratory:
- IDS gene mutation
Also see Idursulfase
Related
Hunter syndrome; mucopolysaccharidosis-2; MPS2
Specific
Idursulfase (iduronate-2-sulfatase)
General
lysosomal hydrolase
sulfatase
Properties
SIZE: entity length = 550 aa
MW = 62 kD
COMPARTMENT: lysosome
MOTIF: signal sequence {1-25}
Ca+2-binding site
SITE: 45-45
Ca+2-binding site
SITE: 46-46
Ca+2-binding site
SITE: 84-84
N-glycosylation site {N115}
N-glycosylation site {N144}
N-glycosylation site {N246}
N-glycosylation site {N280}
N-glycosylation site {N325}
Ca+2-binding site
SITE: 334-334
Ca+2-binding site
SITE: 335-335
N-glycosylation site {N513}
N-glycosylation site {N537}
Database Correlations
OMIM 309900
UniProt P22304
Pfam PF00884
ENZYME 3.1.6.13
References
- UniProt :accession P22304
- Prescriber's Letter 14(2): 2007
New drugs approved by the FDA in 2006
Detail-Document#: 230213
(subscription needed) http://www.prescribersletter.com
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=IDS