IDS

Protein identityUniProt · HPA

UniProt accession

P22304

Gene

IDS

Ensembl

ENSG00000010404

Chromosome

X

Canonical length

550 aa

Protein class

Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted intracellular proteins

Secretome location

Intracellular and membrane

OverviewNCBI Gene

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. Mutations in this gene are associated with the X-linked lysosomal storage disease mucopolysaccharidosis type II, also known as Hunter syndrome. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]

Canonical amino-acid sequenceUniProt

550 residues, UniProt reviewed canonical sequence.

>P22304|IDS
     1  MPPPRTGRGL LWLGLVLSSV CVALGSETQA NSTTDALNVL LIIVDDLRPS LGCYGDKLVR
    61  SPNIDQLASH SLLFQNAFAQ QAVCAPSRVS FLTGRRPDTT RLYDFNSYWR VHAGNFSTIP
   121  QYFKENGYVT MSVGKVFHPG ISSNHTDDSP YSWSFPPYHP SSEKYENTKT CRGPDGELHA
   181  NLLCPVDVLD VPEGTLPDKQ STEQAIQLLE KMKTSASPFF LAVGYHKPHI PFRYPKEFQK
   241  LYPLENITLA PDPEVPDGLP PVAYNPWMDI RQREDVQALN ISVPYGPIPV DFQRKIRQSY
   301  FASVSYLDTQ VGRLLSALDD LQLANSTIIA FTSDHGWALG EHGEWAKYSN FDVATHVPLI
   361  FYVPGRTASL PEAGEKLFPY LDPFDSASQL MEPGRQSMDL VELVSLFPTL AGLAGLQVPP
   421  RCPVPSFHVE LCREGKNLLK HFRFRDLEED PYLPGNPREL IAYSQYPRPS DIPQWNSDKP
   481  SLKDIKIMGY SIRTIDYRYT VWVGFNPDEF LANFSDIHAG ELYFVDSDPL QDHNMYNDSQ
   541  GGDLFQLLMP

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against IDS can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.24

Highest tissue expression

432 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about IDS.

Disease | AllUniProt

Conditions IDS is implicated in, by any mechanism.

• Mucopolysaccharidosis 2 (MPS2) MIM:309900

Disease | GeneticClinVar

796 pathogenic / likely-pathogenic of 1,505 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mucopolysaccharidosis, MPS-II
• Thyroid cancer, nonmedullary, 1
• Inborn genetic diseases
• Nonpapillary renal cell carcinoma
• Mucopolysaccharidosis, MPS-III-A

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.18

gnomAD pLI

1

gnomAD missense Z

1.58

DepMap mean gene effect

-0.01

DepMap dependency class

none

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 6% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• dermatan sulfate proteoglycan catabolic process
• glycosaminoglycan catabolic process
• heparan sulfate proteoglycan catabolic process

Molecular functions

• calcium ion binding
• iduronate-2-sulfatase activity

Cellular components

• lysosomal lumen
• lysosome

Protein domainsUniProt · Pfam · InterPro

• Sulfatase, N-terminal
• Alkaline-phosphatase-like, core domain superfamily
• Sulfatase, conserved site
• Sulfatase
• Iduronate-2-sulfatase

KeywordsUniProt

• Calcium
• Disulfide bond
• Glycoprotein
• Hydrolase
• Lysosome
• Metal-binding
• Mucopolysaccharidosis
• Signal
• Zymogen