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Iduronate 2-sulfatase
Symbols IDS ; MPS2; SIDS
External IDs OMIM300823 MGI96417 HomoloGene169 GeneCards: IDS Gene
EC number
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.[1]


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