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Gene

GNS

glucosamine (N-acetyl)-6-sulfatase

Summary

Cytogenetic location
12q14.3
Coordinates
Chromosome 12: 64,713,449–64,759,406 (GRCh38)
Exons
14 exons across 46 kb
Protein
552 amino acids
Inheritance
Autosomal recessive
MANE Select transcript
ENST00000258145.8NM_002076.4
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 III (Sanfilippo Syndrome)

    • Mucopolysaccharidosis type III (Sanfilippo syndrome types A, B, C and D)Autosomal recessive

      Deficiency of one of four enzymes that break down heparan sulfate (SGSH in type A, NAGLU in type B, HGSNAT in type C, GNS in type D) causes speech delay, hyperactivity, sleep problems and progressive loss of skills in childhood.

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

      There is no approved disease-modifying therapy yet; a genetic diagnosis guides supportive care, family testing and eligibility for clinical trials.

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
      • 6 GenCC submitters rate it definitive or strong

Inheritance

Autosomal recessive
It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.

How GNS is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 12, with a bracket where GNS sits. GNS and 8 genes near it on chromosome 12, from 64,236,428 to 65,236,427.Chromosome 1212q14.3p13.33p13.32p13.31p13.2p13.1p12.3p12.2p12.1p11.23p11.22p11.21p11.1q11q12q13.11q13.12q13.13q13.2q13.3q14.1q14.2q14.3q15q21.1q21.2q21.31q21.32q21.33q22q23.1q23.2q23.3q24.11q24.12q24.13q24.21q24.22q24.23q24.31q24.32q24.3364.4 Mb64.6 Mb64.8 Mb65 Mb65.2 MbC12orf56: 64,264,762–64,390,781, reverse strandC12orf56XPOT: 64,404,168–64,451,130, forward strandXPOTTBK1: 64,452,090–64,502,116, forward strandTBK1RASSF3: 64,507,001–64,697,567, forward strandRASSF3GNS: 64,713,449–64,759,406, reverse strandGNSTBC1D30: 64,759,484–64,881,033, forward strandTBC1D30: 64,759,496–64,977,509, forward strandWIF1: 65,050,565–65,121,582, reverse strandWIF1LEMD3: 65,169,342–65,248,485, forward strandLEMD3Diagram of GNS: 14 exons on the reverse strand of chromosome 12Exon 13: 1 known disease-causing siteExon 12: 4 known disease-causing sitesExon 11: 7 known disease-causing sitesExon 10: 3 known disease-causing sitesExon 9: 4 known disease-causing sitesExon 8: 5 known disease-causing sitesExon 7: 3 known disease-causing sitesExon 6: 5 known disease-causing sitesExon 5: 4 known disease-causing sitesExon 4: 1 known disease-causing siteExon 3: 13 known disease-causing sitesExon 2: 2 known disease-causing sitesExon 1: 6 known disease-causing sitesExon 14: 64,713,449–64,716,819, 3,371 basesExon 13: 64,720,022–64,720,182, 161 basesExon 12: 64,721,595–64,721,705, 111 basesExon 11: 64,723,006–64,723,113, 108 basesExon 10: 64,728,956–64,729,057, 102 basesExon 9: 64,737,004–64,737,107, 104 basesExon 8: 64,739,381–64,739,499, 119 basesExon 7: 64,740,606–64,740,688, 83 basesExon 6: 64,743,141–64,743,308, 168 basesExon 5: 64,744,809–64,744,907, 99 basesExon 4: 64,745,659–64,745,724, 66 basesExon 3: 64,747,712–64,747,918, 207 basesExon 2: 64,752,698–64,752,757, 60 basesExon 1: 64,759,085–64,759,406, 322 bases14131211109876543215′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 5.1 kb of the gene's 46 kb; 1,659 of those bases code for the protein.

How a genome test reads GNS

74

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

None of them falls in a region short reads find hard.

A whole genome covers all 46 kb of GNS, introns included, not only the 5.1 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.). GNS gene: glucosamine (N-acetyl)-6-sulfatase. Retrieved 16 October 2026, from https://dantelabs.com/genes/gns

Vancouver

Dante Labs. GNS gene: glucosamine (N-acetyl)-6-sulfatase [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/gns

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