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Gene

SRY

sex determining region Y

Summary

Cytogenetic location
Yp11.2
Coordinates
Chromosome Y: 2,786,855–2,787,682 (GRCh38)
Exons
1 exon across 0.8 kb
Protein
204 amino acids
Inheritance
Autosomal dominant
Also known as
TDF
MANE Select transcript
ENST00000383070.2NM_003140.3
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Curated reports relevant to this gene

  • 46,XX Differences of Sex Development

    • Conditions including XX testicular and ovotesticular differences of sex development, most often caused by a translocated SRY gene or changes at SOX9 or SOX3, and less often by variants in NR5A1, RSPO1 or WNT4.

      • 2 GenCC submitters rate it definitive or strong
      What knowing changes

      Diagnosis explains azoospermia, informs hormone management, and in RSPO1-related disease prompts skin cancer surveillance.

  • 46,XY Differences of Sex Development and Gonadal Dysgenesis

    • 46,XY complete gonadal dysgenesis (Swyer syndrome)

      A condition in which people with XY chromosomes have undeveloped streak gonads and a female appearance, caused by variants in SRY, NR5A1, MAP3K1, DHH and other genes.

      • 2 GenCC submitters rate it definitive or strong
      What knowing changes

      Streak gonads carry a high risk of gonadoblastoma, so removal is usually recommended, and hormone replacement is needed for puberty.

Broader groupings

  • Hypertrophic Cardiomyopathy

    • Autosomal dominant

      One of 62 genes linked to it

      A condition in which the myocardium is hypertrophied without an obvious cause. The hypertrophy is generally asymmetric and may be associated with obstruction of the ventricular outflow tract.

      • 2 GenCC submitters rate it definitive or strong
      What knowing changes

      Beta-blockers, non-dihydropyridine calcium channel blockers, activity restriction, genetic counseling

Inheritance

Autosomal dominant
One changed copy, from either parent, is enough. A parent who has one passes it on to each child with a one-in-two chance.

How SRY is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
Chromosome Y, with a bracket where SRY sits. SRY and 5 genes near it on chromosome Y, from 2,287,269 to 3,287,268.Chromosome YYp11.2p11.32p11.31p11.2p11.1q11.1q11.21q11.221q11.222q11.223q11.23q122.4 Mb2.6 Mb2.8 Mb3 Mb3.2 MbDHRSX: 2,217,548–2,502,805, reverse strandDHRSXZBED1: 2,486,169–2,502,862, reverse strandZBED1CD99: 2,690,988–2,741,312, forward strandCD99SRY: 2,786,855–2,787,682, reverse strandSRYRPS4Y1: 2,841,402–2,932,000, forward strandRPS4Y1ZFY: 2,935,201–2,982,579, forward strandZFYDiagram of SRY: one exon on the reverse strand of chromosome YExon 1: 15 known disease-causing sitesExon 1: 2,786,855–2,787,682, 828 bases15′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 0.8 kb of the gene's 0.8 kb; 615 of those bases code for the protein.

How a genome test reads SRY

15

positions in SRY 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 0.8 kb of SRY, introns included, not only the 0.8 kb of exons.

Genes linked to the same conditions

Traits linked to SRY

The Human Phenotype Ontology lists these as features of the rare conditions SRY can cause. Each trait's page lists the other genes linked to it.

Abnormality of the genitourinary system

Show 48 more traits

External links

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.). SRY gene: sex determining region Y. Retrieved 17 October 2026, from https://dantelabs.com/genes/sry

Vancouver

Dante Labs. SRY gene: sex determining region Y [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/sry

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