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Gene

PRKAR1A

protein kinase cAMP-dependent type I regulatory subunit alpha

Summary

Cytogenetic location
17q24.2
Coordinates
Chromosome 17: 68,512,430–68,533,431 (GRCh38)
Exons
11 exons across 21 kb
Protein
381 amino acids
Inheritance
Autosomal dominant
Also known as
CNC1
Previous symbols
PRKAR1, TSE1, PKR1, Prkar1alpha
MANE Select transcript
ENST00000589228.6NM_002734.5
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

  • Carney Complex & Hereditary Adrenal Cushing Syndrome

    • Carney complexAutosomal dominant

      A multiple neoplasia syndrome with spotty skin pigmentation, cardiac and skin myxomas, and endocrine tumours of the adrenal, pituitary, thyroid and testes.

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

      Regular heart ultrasound to find myxomas early, plus endocrine and testicular surveillance, and testing of relatives.

    • Primary pigmented nodular adrenocortical diseaseAutosomal dominant

      Small pigmented adrenal nodules that produce cortisol independently of ACTH, causing Cushing syndrome, either alone or as part of Carney complex.

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

      Biochemical screening for cortisol excess allows Cushing syndrome to be recognised and treated early.

  • Pseudohypoparathyroidism & GNAS-Inactivation Disorders

    • AcrodysostosisAutosomal dominant

      A skeletal condition with short hands and feet and facial differences, sometimes with hormone resistance, caused by PRKAR1A or PDE4D variants.

      • 4 GenCC submitters rate it definitive or strong

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 PRKAR1A is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
Chromosome 17, with a bracket where PRKAR1A sits. PRKAR1A and 8 genes near it on chromosome 17, from 68,022,931 to 69,022,930.Chromosome 1717q24.2p13.3p13.2p13.1p12p11.2p11.1q11.1q11.2q12q21.1q21.2q21.31q21.32q21.33q22q23.1q23.2q23.3q24.1q24.2q24.3q25.1q25.2q25.368.2 Mb68.4 Mb68.6 Mb68.8 Mb69 MbKPNA2: 68,035,517–68,047,364, forward strandKPNA2AMZ2: 68,205,431–68,258,828, forward strandAMZ2ARSG: 68,259,170–68,422,731, forward strandARSGSLC16A6: 68,267,026–68,291,267, reverse strandSLC16A6WIPI1: 68,420,948–68,457,563, reverse strandWIPI1PRKAR1A: 68,512,430–68,533,431, forward strandPRKAR1AFAM20A: 68,535,113–68,601,407, reverse strandFAM20AABCA8: 68,866,052–68,955,470, reverse strandABCA8ABCA9: 68,974,488–69,061,087, reverse strandABCA9Diagram of PRKAR1A: 11 exons on the forward strand of chromosome 17Exon 1: 2 known disease-causing sitesExon 2: 6 known disease-causing sitesExon 3: 8 known disease-causing sitesExon 4: 2 known disease-causing sitesExon 5: 4 known disease-causing sitesExon 6: 3 known disease-causing sitesExon 7: 8 known disease-causing sitesExon 8: 4 known disease-causing sitesExon 9: 7 known disease-causing sitesExon 10: 5 known disease-causing sitesExon 11: 7 known disease-causing sitesExon 1: 68,512,430–68,512,548, 119 basesExon 2: 68,515,394–68,515,576, 183 basesExon 3: 68,522,756–68,522,926, 171 basesExon 4: 68,523,725–68,523,816, 92 basesExon 5: 68,524,016–68,524,077, 62 basesExon 6: 68,524,912–68,524,958, 47 basesExon 7: 68,525,754–68,525,912, 159 basesExon 8: 68,527,840–68,527,900, 61 basesExon 9: 68,528,870–68,528,991, 122 basesExon 10: 68,529,920–68,530,001, 82 basesExon 11: 68,530,277–68,533,431, 3,155 bases1246810115′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 4.3 kb of the gene's 21 kb; 1,146 of those bases code for the protein.

How a genome test reads PRKAR1A

72

positions in PRKAR1A 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 21 kb of PRKAR1A, introns included, not only the 4.3 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.). PRKAR1A gene: protein kinase cAMP-dependent type I regulatory subunit alpha. Retrieved 16 October 2026, from https://dantelabs.com/genes/prkar1a

Vancouver

Dante Labs. PRKAR1A gene: protein kinase cAMP-dependent type I regulatory subunit alpha [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/prkar1a

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