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Gene

IDS

iduronate 2-sulfatase

Summary

Cytogenetic location
Xq28
Coordinates
Chromosome X: 149,476,988–149,505,306 (GRCh38)
Exons
9 exons across 28.3 kb
Protein
550 amino acids
Inheritance
X-linked recessive
Also known as
ID2S
Previous symbols
SIDS
MANE Select transcript
ENST00000340855.11NM_000202.8
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • Mucopolysaccharidosis Type II (Hunter Syndrome)

    • X-linked recessive

      Deficiency of iduronate-2-sulfatase causes glycosaminoglycan storage with coarse features, enlarged liver and spleen, joint stiffness, airway and heart disease and, in the severe form, cognitive decline.

      • ClinGen rates the link definitive
      • 7 GenCC submitters rate it definitive or strong
      What knowing changes

      Enzyme replacement therapy (idursulfase) is available, and identifying carrier women allows family testing and reproductive planning.

Broader groupings

  • Lysosomal Storage Disease

    • Lysosomal storage disease (other and unspecified types)

      One of 59 genes linked to it

      Covers lysosomal storage diseases without a dedicated report, such as Farber disease and other ASAH1-related disorders, prosaposin (PSAP) deficiencies, multiple sulfatase deficiency, mucopolysaccharidosis types IX and X, and Dorfman-Chanarin disease; inheritance varies by subtype, most often autosomal recessive.

      • ClinGen rates the link definitive
      • 7 GenCC submitters rate it definitive or strong

Inheritance

X-linked recessive
The change is on the X chromosome, so one copy affects males, who have one X, while females with one are usually carriers. A carrier mother passes it on to each child with a one-in-two chance.

How IDS is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome X, with a bracket where IDS sits. IDS and 14 genes near it on chromosome X, from 148,991,147 to 149,991,146.Chromosome XXq28p22.33p22.32p22.31p22.2p22.13p22.12p22.11p21.3p21.2p21.1p11.4p11.3p11.23p11.22p11.21p11.1q11.1q11.2q12q13.1q13.2q13.3q21.1q21.2q21.31q21.32q21.33q22.1q22.2q22.3q23q24q25q26.1q26.2q26.3q27.1q27.2q27.3q28149 Mb149.2 Mb149.4 Mb149.6 Mb149.8 MbAFF2: 148,500,611–149,000,663, forward strandAFF2: 149,414,877–149,415,506, reverse strandIDS: 149,476,988–149,505,306, reverse strandIDS: 149,477,104–149,540,926, reverse strandEOLA1: 149,536,605–149,555,341, forward strandEOLA1HSFX3: 149,548,210–149,549,924, reverse strandHSFX3MAGEA9B: 149,581,653–149,587,471, reverse strandMAGEA9BHSFX2: 149,592,512–149,595,314, reverse strandHSFX2TMEM185A: 149,596,551–149,631,912, reverse strandTMEM185AMAGEA11: 149,688,228–149,717,268, forward strandMAGEA11HSFX1: 149,774,068–149,776,867, forward strandHSFX1MAGEA9: 149,781,930–149,787,737, forward strandMAGEA9MAGEA8: 149,881,141–149,885,835, forward strandMAGEA8EOLA2: 149,921,940–149,938,881, reverse strandEOLA2HSFX4: 149,929,573–149,931,287, forward strandHSFX4Diagram of IDS: 9 exons on the reverse strand of chromosome XExon 9: 132 known disease-causing sitesExon 8: 55 known disease-causing sitesExon 7: 34 known disease-causing sitesExon 6: 50 known disease-causing sitesExon 5: 59 known disease-causing sitesExon 4: 34 known disease-causing sitesExon 3: 82 known disease-causing sitesExon 2: 49 known disease-causing sitesExon 1: 23 known disease-causing sitesExon 9: 149,476,988–149,483,218, 6,231 basesExon 8: 149,486,925–149,487,098, 174 basesExon 7: 149,490,314–149,490,440, 127 basesExon 6: 149,496,346–149,496,516, 171 basesExon 5: 149,498,107–149,498,307, 201 basesExon 4: 149,500,949–149,501,037, 89 basesExon 3: 149,503,312–149,503,489, 178 basesExon 2: 149,504,157–149,504,293, 137 basesExon 1: 149,505,035–149,505,306, 272 bases9876543215′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 7.6 kb of the gene's 28.3 kb; 1,653 of those bases code for the protein.

How a genome test reads IDS

558

positions in IDS where ClinVar classes a change as pathogenic or likely pathogenic

115 of them (21%) fall in regions short reads find hard.

  • Low mappability (90 sites)Regions where 100 bp or 250 bp reads cannot be placed uniquely.
  • Segmental duplications (115 sites)Copies of at least 1 kb sharing at least 90% identity with another part of the genome (paralogues, pseudogenes); reads may align to the wrong copy.

A whole genome covers all 28.3 kb of IDS, introns included, not only the 7.6 kb of exons.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). IDS gene: iduronate 2-sulfatase. Retrieved 16 October 2026, from https://dantelabs.com/genes/ids

Vancouver

Dante Labs. IDS gene: iduronate 2-sulfatase [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/ids

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