Iduronate-2-sulfatase

Iduronate 2-sulfatase
Identifiers
Symbols	IDS ; MPS2; SIDS
External IDs	OMIM: 300823 MGI: 96417 HomoloGene: 169 GeneCards: IDS Gene
EC number	3.1.6.13
Gene ontology Molecular function • iduronate-2-sulfatase activity • metal ion binding Cellular component • lysosomal lumen Biological process • carbohydrate metabolic process • glycosaminoglycan catabolic process • glycosaminoglycan metabolic process • chondroitin sulfate metabolic process • chondroitin sulfate catabolic process • small molecule metabolic process Sources: Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	3423	15931
Ensembl	ENSG00000010404	ENSMUSG00000035847
UniProt	P22304	Q08890
RefSeq (mRNA)	NM_000202	NM_001038990
RefSeq (protein)	NP_000193	NP_034628
Location (UCSC)	Chr X: 149.48 – 149.52 Mb	Chr X: 70.34 – 70.37 Mb
PubMed search	[1]	[2]

Iduronate 2-sulfatase is a sulfatase enzyme associated with Hunter syndrome.

Iduronate 2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-chromosome gene that result in enzymatic deficiency lead to the sex-linked mucopolysaccharidosis type II, also known as Hunter syndrome. Iduronate-2-sulfatase has a strong sequence homology with human arylsulfatases A, B, and C, and human glucosamine-6-sulfatase. A splice variant of this gene has been described.

External links

• GeneReviews/NIH/NCBI/UW entry on Mucopolysaccharidosis Type II

See also

• Idursulfase