Skip to content
Use your HSA or FSA funds

Gene

PROS1

protein S

Summary

Cytogenetic location
3q11.1
Coordinates
Chromosome 3: 93,873,051–93,973,896 (GRCh38)
Exons
15 exons across 100.8 kb
Protein
676 amino acids
Inheritance
Autosomal dominant
Previous symbols
PROS
MANE Select transcript
ENST00000394236.9NM_000313.4
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 report relevant to this gene

  • Thrombophilia

    • Protein S DeficiencyAutosomal dominant

      An inherited shortage of the natural anticoagulant protein S, caused by changes in PROS1, that raises the risk of blood clots in the veins.

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

      Knowing about it informs decisions about clot prevention around surgery, pregnancy and oestrogen-containing medicines.

    • Inherited Thrombophilia
      • 4 GenCC submitters rate it definitive or strong
      What knowing changes

      Anticoagulation (duration by genotype); avoid estrogen-containing contraceptives; LMWH for pregnancy prophylaxis; extended post-surgical anticoagulation; lifelong anticoagulation for compound/high-risk genotypes

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 PROS1 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 3, with a bracket where PROS1 sits. PROS1 and 4 genes near it on chromosome 3, from 93,423,474 to 94,423,473.Chromosome 33q11.1p26.3p26.2p26.1p25.3p25.2p25.1p24.3p24.2p24.1p23p22.3p22.2p22.1p21.33p21.32p21.31p21.2p21.1p14.3p14.2p14.1p13p12.3p12.2p12.1p11.2p11.1q11.1q11.2q12.1q12.2q12.3q13.11q13.12q13.13q13.2q13.31q13.32q13.33q21.1q21.2q21.3q22.1q22.2q22.3q23q24q25.1q25.2q25.31q25.32q25.33q26.1q26.2q26.31q26.32q26.33q27.1q27.2q27.3q28q2993.6 Mb93.8 Mb94 Mb94.2 Mb94.4 MbPROS1: 93,873,051–93,973,896, reverse strandPROS1ARL13B: 93,980,128–94,055,678, forward strandARL13BDHFR2: 93,981,180–94,063,389, reverse strandDHFR2STX19: 94,012,043–94,028,658, reverse strandSTX19NSUN3: 94,062,980–94,131,832, forward strandNSUN3Diagram of PROS1: 15 exons on the reverse strand of chromosome 3Exon 15: 10 known disease-causing sitesExon 14: 7 known disease-causing sitesExon 13: 5 known disease-causing sitesExon 12: 8 known disease-causing sitesExon 11: 7 known disease-causing sitesExon 10: 11 known disease-causing sitesExon 9: 3 known disease-causing sitesExon 8: 11 known disease-causing sitesExon 7: 3 known disease-causing sitesExon 6: 8 known disease-causing sitesExon 5: 3 known disease-causing sitesExon 4: 3 known disease-causing sitesExon 3: 1 known disease-causing siteExon 2: 10 known disease-causing sitesExon 1: 4 known disease-causing sitesExon 15: 93,873,051–93,874,405, 1,355 basesExon 14: 93,876,966–93,877,191, 226 basesExon 13: 93,879,163–93,879,314, 152 basesExon 12: 93,884,728–93,884,896, 169 basesExon 11: 93,886,336–93,886,503, 168 basesExon 10: 93,892,933–93,893,122, 190 basesExon 9: 93,896,576–93,896,691, 116 basesExon 8: 93,898,448–93,898,569, 122 basesExon 7: 93,900,804–93,900,929, 126 basesExon 6: 93,905,784–93,905,915, 132 basesExon 5: 93,906,021–93,906,143, 123 basesExon 4: 93,910,619–93,910,705, 87 basesExon 3: 93,924,240–93,924,264, 25 basesExon 2: 93,927,250–93,927,407, 158 basesExon 1: 93,973,674–93,973,896, 223 bases15141210864215′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 3.4 kb of the gene's 100.8 kb; 2,031 of those bases code for the protein.

How a genome test reads PROS1

117

positions in PROS1 where ClinVar classes a change as pathogenic or likely pathogenic

111 of them (95%) fall in regions short reads find hard.

  • Low mappability (40 sites)Regions where 100 bp or 250 bp reads cannot be placed uniquely.
  • Segmental duplications (111 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 100.8 kb of PROS1, introns included, not only the 3.4 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.). PROS1 gene: protein S. Retrieved 17 October 2026, from https://dantelabs.com/genes/pros1

Vancouver

Dante Labs. PROS1 gene: protein S [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/pros1

One test. A lifetime of answers.

From $449Free shipping · Results in 6–8 weeks

Get my Genome Test