• Gene id: 3423

Gene Summary

• Gene Symbol: IDS   Gene   UCSC   Ensembl
• Aliases: ID2S, MPS2, SIDS
• Gene name: iduronate 2-sulfatase
• Alternate names: iduronate 2-sulfatase, alpha-L-iduronate sulfate sulfatase, iduronate 2-sulfatase 14 kDa chain, iduronate 2-sulfatase 42 kDa chain, idursulfase,
• Gene location: Xq28 (9475512: 9538113)     Exons: 10     NC_000018.10
• Gene summary(Entrez): This gene encodes a member of the sulfatase family of proteins. The encoded preproprotein is proteolytically processed to generate two polypeptide chains. This enzyme is involved in the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutati
• OMIM: 300823

Protein Summary

• Protein general information: P22304  
  • Name: Iduronate 2 sulfatase (EC 3.1.6.13) (Alpha L iduronate sulfate sulfatase) (Idursulfase) [Cleaved into: Iduronate 2 sulfatase 42 kDa chain; Iduronate 2 sulfatase 14 kDa chain]
  • Length: 550  
  • Mass: 61873
  • Tissue specificity: Liver, kidney, lung, and placenta.
• Sequence:

  MPPPRTGRGLLWLGLVLSSVCVALGSETQANSTTDALNVLLIIVDDLRPSLGCYGDKLVRSPNIDQLASHSLLFQ
  NAFAQQAVCAPSRVSFLTGRRPDTTRLYDFNSYWRVHAGNFSTIPQYFKENGYVTMSVGKVFHPGISSNHTDDSP
  YSWSFPPYHPSSEKYENTKTCRGPDGELHANLLCPVDVLDVPEGTLPDKQSTEQAIQLLEKMKTSASPFFLAVGY
  HKPHIPFRYPKEFQKLYPLENITLAPDPEVPDGLPPVAYNPWMDIRQREDVQALNISVPYGPIPVDFQRKIRQSY
  FASVSYLDTQVGRLLSALDDLQLANSTIIAFTSDHGWALGEHGEWAKYSNFDVATHVPLIFYVPGRTASLPEAGE
  KLFPYLDPFDSASQLMEPGRQSMDLVELVSLFPTLAGLAGLQVPPRCPVPSFHVELCREGKNLLKHFRFRDLEED
  PYLPGNPRELIAYSQYPRPSDIPQWNSDKPSLKDIKIMGYSIRTIDYRYTVWVGFNPDEFLANFSDIHAGELYFV
  DSDPLQDHNMYNDSQGGDLFQLLMP

• Structural information:
  • Interpro: IPR017850  IPR035874  IPR024607  IPR000917  
  • Prosite: PS00523 PS00149
  • CDD: cd16030
  • PDB: 5FQL 6IOZ
  • PDBsum: 5FQL 6IOZ
  • STRING: ENSP00000339801
• Other Databases: GeneCards:  IDS  Malacards:  IDS

Gene ontology

GO accession	Term name	Evidence code	Go category
GO:0004423	iduronate-2-sulfatase activity	IBA	molecular function
GO:0008484	sulfuric ester hydrolase activity	IBA	molecular function
GO:0004423	iduronate-2-sulfatase activity	IDA	molecular function
GO:0005764	lysosome	IDA	cellular component
GO:0006027	glycosaminoglycan catabolic process	IDA	biological process
GO:0005509	calcium ion binding	IDA	molecular function
GO:0004423	iduronate-2-sulfatase activity	IEA	molecular function
GO:0003824	catalytic activity	IEA	molecular function
GO:0008484	sulfuric ester hydrolase activity	IEA	molecular function
GO:0016787	hydrolase activity	IEA	molecular function
GO:0046872	metal ion binding	IEA	molecular function
GO:0005764	lysosome	IEA	cellular component
GO:0004423	iduronate-2-sulfatase activity	TAS	molecular function
GO:0004423	iduronate-2-sulfatase activity	IEA	molecular function
GO:0043202	lysosomal lumen	TAS	cellular component
GO:0043202	lysosomal lumen	TAS	cellular component
GO:0006027	glycosaminoglycan catabolic process	TAS	biological process
GO:0030207	chondroitin sulfate catabolic process	TAS	biological process
GO:0005764	lysosome	IEA	cellular component

KEGG pathways

Pathway id	Pathway name
hsa01100	Metabolic pathways
hsa04142	Lysosome
hsa00531	Glycosaminoglycan degradation

Diseases

Associated diseases	References
Mucopolysaccharidosis	KEGG:H00421
Mucopolysaccharidosis type II	KEGG:H00129
Mucopolysaccharidosis	KEGG:H00421
Mucopolysaccharidosis type II	KEGG:H00129
mucopolysaccharidosis II	PMID:27146977
mucopolysaccharidosis II	PMID:1550586
Aberrant CpGs in Low Motility Sperm	MIK: 21674046
Cryptorchidism	MIK: 28606200
Teratozoospermia	MIK: 17327269

PubMed references

PMID	Condition	Mutation	Ethnicity	Population details	Infertility_type	Associated_genes
17327269	Teratozoospermia			13 (5 controls, 8 cases)	Male infertility	GSE6967 analyzed by GEO2R (cutoff 1.5 fold)
21674046	Aberrant CpGs in Low Motility Sperm			18	Male infertility	GSE26881
28606200	Cryptorchidism			Monozgotic twins (1 control, I cwith cryptorchidism)	Male infertility	MeDIP-Seq