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Gene

PRKG1

protein kinase cGMP-dependent 1

Summary

Cytogenetic location
10q11.23-q21.1
Coordinates
Chromosome 10: 51,074,487–52,298,350 (GRCh38)
Exons
18 exons across 1,223.9 kb
Protein
686 amino acids
Inheritance
Autosomal dominant
Also known as
PGK, PKG, PKG1
Previous symbols
PRKGR1B, PRKG1B
MANE Select transcript
ENST00000373980.11NM_006258.4
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

  • Aortic Aneurysm

    • Familial Thoracic Aortic Aneurysm and DissectionAutosomal dominant

      Inherited widening and tearing of the thoracic aorta, caused by changes in genes such as ACTA2, MYH11, MYLK, LOX, PRKG1, MFAP5 and THSD4.

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

      Knowing about it supports regular aortic imaging, blood pressure control and timely preventive surgery, and screening of relatives.

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 PRKG1 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 10, with a bracket where PRKG1 sits. PRKG1 and 12 genes near it on chromosome 10, from 49,850,623 to 53,522,214.Chromosome 1010q11.23-q21.1p15.3p15.2p15.1p14p13p12.33p12.32p12.31p12.2p12.1p11.23p11.22p11.21p11.1q11.1q11.21q11.22q11.23q21.1q21.2q21.3q22.1q22.2q22.3q23.1q23.2q23.31q23.32q23.33q24.1q24.2q24.31q24.32q24.33q25.1q25.2q25.3q26.11q26.12q26.13q26.2q26.350 Mb50.5 Mb51 Mb51.5 Mb52 Mb52.5 Mb53 Mb53.5 MbPARG: 49,815,132–49,970,203, reverse strandTIMM23B: 49,942,031–49,977,625, forward strandTIMM23BTIMM23B-AGAP6: 49,942,056–50,011,654, forward strandTIMM23B-AGAP6AGAP6: 49,982,190–50,011,654, forward strandAGAP6WASHC2A: 50,067,881–50,136,578, forward strandWASHC2AASAH2: 50,182,778–50,279,720, reverse strandASAH2SGMS1: 50,305,586–50,625,189, reverse strandSGMS1ASAH2B: 50,739,318–50,816,495, forward strandASAH2BA1CF: 50,799,409–50,885,716, reverse strandA1CFPRKG1: 51,074,487–52,298,350, forward strandPRKG1CSTF2T: 51,695,486–51,699,595, reverse strandCSTF2TDKK1: 52,314,274–52,318,042, forward strandDKK1MBL2: 52,758,075–52,773,009, reverse strandMBL2Diagram of PRKG1: 18 exons on the forward strand of chromosome 10Exon 3: 1 known disease-causing siteExon 1: 51,074,487–51,074,901, 415 basesExon 2: 51,153,164–51,153,330, 167 basesExon 3: 51,467,723–51,467,836, 114 basesExon 4: 51,804,585–51,804,690, 106 basesExon 5: 51,907,507–51,907,570, 64 basesExon 6: 52,054,484–52,054,561, 78 basesExon 7: 52,062,537–52,062,631, 95 basesExon 8: 52,133,840–52,133,905, 66 basesExon 9: 52,161,889–52,161,963, 75 basesExon 10: 52,251,570–52,251,666, 97 basesExon 11: 52,271,350–52,271,489, 140 basesExon 12: 52,272,392–52,272,481, 90 basesExon 13: 52,280,789–52,280,930, 142 basesExon 14: 52,282,153–52,282,316, 164 basesExon 15: 52,288,726–52,288,848, 123 basesExon 16: 52,288,931–52,288,993, 63 basesExon 17: 52,290,224–52,290,290, 67 basesExon 18: 52,293,802–52,298,350, 4,549 bases110185′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 6.6 kb of the gene's 1,223.9 kb; 2,061 of those bases code for the protein.

How a genome test reads PRKG1

1

position in PRKG1 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 1,223.9 kb of PRKG1, introns included, not only the 6.6 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.). PRKG1 gene: protein kinase cGMP-dependent 1. Retrieved 16 October 2026, from https://dantelabs.com/genes/prkg1

Vancouver

Dante Labs. PRKG1 gene: protein kinase cGMP-dependent 1 [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/prkg1

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