IDS - Iduronate 2-sulfatase - human protein (Function)
 
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Protein
Gene
References

 
IDS »  Iduronate 2-sulfatase   [ EC 3.1.6.13 ]
 
Protein also known as:  Alpha-L-iduronate sulfate sulfatase (Idursulfase). Cleaved into:  Iduronate 2-sulfatase 14 kDa chain; Iduronate 2-sulfatase 42 kDa chain.
Gene name:  IDS
Family name: Sulfatase
Entry whose protein(s) existence is based on evidence at protein level
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GENE REF ISO

Function

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Overview 
Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.  
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  • UniProtKB
GO molecular function 
Metal ion bindingdefinition[GO:0046872]  
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  • UniProtKB KW
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 3.1.6.13: Hydrolysis of the 2-sulfate groups of the L-iduronate 2-sulfate units of dermatan sulfate, heparan sulfate and heparin.  
1
  • UniProtKB
It requires the following cofactor
 Ca(2+) : Binds 1 Ca(2+) ion per subunit.  
1
  • UniProtKB
 
More information is available from:
Pathways 
According to KEGG, this protein belongs to the following pathways:
Glycosaminoglycan degradation  hsa00531+3423  
Lysosome  hsa04142+3423  
Metabolic pathways  hsa01100+3423  
According to Reactome, this protein belongs to the following pathways:
CS/DS degradation  REACT_120888  
HS-GAG degradation  REACT_120752  
MPS II - Hunter syndrome  REACT_147734  
 
More information is available from:
 

Keywords

Molecular function 
Hydrolase  definition   [KW-0378]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P22304
Other
GeneWiki: Iduronate-2-sulfatase
GenomeRNAi: 3423
PRO: PR:P22304