PRKAR1A

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

PBB_Summary
section_title =
summary_text = 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. [cite web | title = Entrez Gene: PRKAR1A protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=5573| accessdate = ]

Mutation of PRKAR1A leads to the Carney complex, associating multiple endocrine tumors.Fact|date=December 2007

ee also

*cAMP-dependent protein kinase

References

Further reading

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

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