PRKAR1A

Protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1)
File:PBB Protein PRKAR1A image.jpg PDB rendering based on 1ne4.
Available structures PDB Ortholog search: Template:Homologene2PDBe PDBe, Template:Homologene2uniprot RCSB List of PDB id codes 1ne4​, 1ne6​, 1rgs​, 1rl3​, 1u7e​, 2ezw​
Identifiers
Symbols	PRKAR1A ; CAR; CNC; CNC1; DKFZp779L0468; MGC17251; PKR1; PRKAR1; TSE1
External IDs	Template:OMIM5 Template:MGI HomoloGene: 37664
Gene ontology Molecular function Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Cellular component Template:GNF GO Template:GNF GO Biological process Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO
RNA expression pattern
File:PBB GE PRKAR1A 200603 at tn.png
File:PBB GE PRKAR1A 200604 s at tn.png
File:PBB GE PRKAR1A 200605 s at tn.png
More reference expression data
Orthologs
Template:GNF Ortholog box
Species	Human	Mouse
Entrez	n/a	n/a
Ensembl	n/a	n/a
UniProt	n/a	n/a
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	n/a	n/a
Location (UCSC)	n/a	n/a
PubMed search	n/a	n/a

Protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1), also known as PRKAR1A, is a human gene.

cAMP is a signaling molecule important for a variety of cellular functions. cAMP exerts its effects by activating the cAMP-dependent protein kinase (AMPK), which transduces the signal through phosphorylation of different target proteins. The inactive holoenzyme of AMPK is a tetramer composed of two regulatory and two catalytic subunits. cAMP causes the dissociation of the inactive holoenzyme into a dimer of regulatory subunits bound to four cAMP and two free monomeric catalytic subunits. Four different regulatory subunits and three catalytic subunits of AMPK have been identified in humans. The protein encoded by this gene is one of the regulatory subunits. This protein was found to be a tissue-specific extinguisher that down-regulates the expression of seven liver genes in hepatoma x fibroblast hybrids. Functional null mutations in this gene cause Carney complex (CNC), an autosomal dominant multiple neoplasia syndrome. This gene can fuse to the RET protooncogene by gene rearrangement and form the thyroid tumor-specific chimeric oncogene known as PTC2. Three alternatively spliced transcript variants encoding the same protein have been observed.

Mutation of PRKAR1A leads to the Carney complex, associating multiple endocrine tumors.

See also

• cAMP-dependent protein kinase

Further reading

• Stratakis CA (2002). "Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit (PRKAR1A) in patients with the "complex of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas" (Carney complex)". Ann. N. Y. Acad. Sci. 968: 3–21. PMID 12119264.
• Bourdeau I, Stratakis CA (2002). "Cyclic AMP-dependent signaling aberrations in macronodular adrenal disease". Ann. N. Y. Acad. Sci. 968: 240–55. PMID 12119280.
• Alto N, Carlisle Michel JJ, Dodge KL; et al. (2003). "Intracellular targeting of protein kinases and phosphatases". Diabetes. 51 Suppl 3: S385–8. PMID 12475780.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Stergiopoulos SG, Stratakis CA (2003). "Human tumors associated with Carney complex and germline PRKAR1A mutations: a protein kinase A disease!". FEBS Lett. 546 (1): 59–64. PMID 12829237.
• Bossis I, Stratakis CA (2004). "Minireview: PRKAR1A: normal and abnormal functions". Endocrinology. 145 (12): 5452–8. doi:10.1210/en.2004-0900. PMID 15331577.
• Cho-Chung YS, Nesterova MV (2006). "Tumor reversion: protein kinase A isozyme switching". Ann. N. Y. Acad. Sci. 1058: 76–86. doi:10.1196/annals.1359.014. PMID 16394127.
• Lytras A, Tolis G (2006). "Growth hormone-secreting tumors: genetic aspects and data from animal models". Neuroendocrinology. 83 (3–4): 166–78. doi:10.1159/000095525. PMID 17047380.
• Harrich D, McMillan N, Munoz L; et al. (2007). "Will diverse Tat interactions lead to novel antiretroviral drug targets?". Current drug targets. 7 (12): 1595–606. PMID 17168834.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Boshart M, Weih F, Nichols M, Schütz G (1991). "The tissue-specific extinguisher locus TSE1 encodes a regulatory subunit of cAMP-dependent protein kinase". Cell. 66 (5): 849–59. PMID 1832337.CS1 maint: Multiple names: authors list (link)
• Jones KW, Shapero MH, Chevrette M, Fournier RE (1991). "Subtractive hybridization cloning of a tissue-specific extinguisher: TSE1 encodes a regulatory subunit of protein kinase A.". Cell. 66 (5): 861–72. PMID 1889088.CS1 maint: Multiple names: authors list (link)
• Hofmann B, Nishanian P, Baldwin RL; et al. (1991). "HIV inhibits the early steps of lymphocyte activation, including initiation of inositol phospholipid metabolism". J. Immunol. 145 (11): 3699–705. PMID 1978848.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Sandberg M, Skålhegg B, Jahnsen T (1990). "The two mRNA forms for the type I alpha regulatory subunit of cAMP-dependent protein kinase from human testis are due to the use of different polyadenylation site signals". Biochem. Biophys. Res. Commun. 167 (1): 323–30. PMID 2310396.CS1 maint: Multiple names: authors list (link)
• Lem J, Chin AC, Thayer MJ; et al. (1988). "Coordinate regulation of two genes encoding gluconeogenic enzymes by the trans-dominant locus Tse-1". Proc. Natl. Acad. Sci. U.S.A. 85 (19): 7302–6. PMID 2902627.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Sandberg M, Taskén K, Oyen O; et al. (1988). "Molecular cloning, cDNA structure and deduced amino acid sequence for a type I regulatory subunit of cAMP-dependent protein kinase from human testis". Biochem. Biophys. Res. Commun. 149 (3): 939–45. PMID 3426618.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Scambler P, Oyen O, Wainwright B; et al. (1987). "Exclusion of catalytic and regulatory subunits of cAMP-dependent protein kinase as candidate genes for the defect causing cystic fibrosis". Am. J. Hum. Genet. 41 (5): 925–32. PMID 3479018.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Geahlen RL, Carmichael DF, Hashimoto E, Krebs EG (1982). "Phosphorylation of cAMP-dependent protein kinase subunits". Adv. Enzyme Regul. 20: 195–209. PMID 6287816.CS1 maint: Multiple names: authors list (link)
• Guild BC, Strominger JL (1984). "HLA-A2 antigen phosphorylation in vitro by cyclic AMP-dependent protein kinase. Sites of phosphorylation and segmentation in class i major histocompatibility complex gene structure". J. Biol. Chem. 259 (21): 13504–10. PMID 6333425.
• Bongarzone I, Monzini N, Borrello MG; et al. (1993). "Molecular characterization of a thyroid tumor-specific transforming sequence formed by the fusion of ret tyrosine kinase and the regulatory subunit RI alpha of cyclic AMP-dependent protein kinase A.". Mol. Cell. Biol. 13 (1): 358–66. PMID 7678053.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Hofmann B, Nishanian P, Nguyen T; et al. (1993). "Human immunodeficiency virus proteins induce the inhibitory cAMP/protein kinase A pathway in normal lymphocytes". Proc. Natl. Acad. Sci. U.S.A. 90 (14): 6676–80. PMID 7688126.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Hofmann B, Nishanian P, Fan J; et al. (1994). "HIV Gag p17 protein impairs proliferation of normal lymphocytes in vitro". AIDS. 8 (7): 1016–7. PMID 7946090.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)

This article incorporates text from the United States National Library of Medicine, which is in the public domain.