PRKAG2

AMP-activated protein kinase (AMPK) is a heterotrimeric protein composed of a catalytic alpha subunit, a noncatalytic beta subunit, and a noncatalytic regulatory gamma subunit. Various forms of each of these subunits exist, encoded by different genes. AMPK is an important energy-sensing enzyme that monitors cellular energy status and functions by inactivating key enzymes involved in regulating de novo biosynthesis of fatty acid and cholesterol. This gene is a member of the AMPK gamma subunit family and encodes a protein with four CBS domains. Mutations in this gene have been associated with ventricular pre-excitation (Wolff–Parkinson–White syndrome), progressive conduction system disease and cardiac hypertrophy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

PRKAG2 has been shown to interact with PRKAB2{{cite journal | vauthors = Cheung PC, Salt IP, Davies SP, Hardie DG, Carling D | title = Characterization of AMP-activated protein kinase gamma-subunit isoforms and their role in AMP binding | journal = The Biochemical Journal | volume = 346 Pt 3 | issue = Pt 3 | pages = 659–669 | date = March 2000 | pmid = 10698692 | pmc = 1220898 | doi = 10.1042/0264-6021:3460659 }} and PRKAB1.

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• {{cite journal | vauthors = Gollob MH, Green MS, Tang AS, Roberts R | title = PRKAG2 cardiac syndrome: familial ventricular preexcitation, conduction system disease, and cardiac hypertrophy | journal = Current Opinion in Cardiology | volume = 17 | issue = 3 | pages = 229–234 | date = May 2002 | pmid = 12015471 | doi = 10.1097/00001573-200205000-00004 | s2cid = 33599152 }}
• {{cite journal | vauthors = Gollob MH | title = Glycogen storage disease as a unifying mechanism of disease in the PRKAG2 cardiac syndrome | journal = Biochemical Society Transactions | volume = 31 | issue = Pt 1 | pages = 228–231 | date = February 2003 | pmid = 12546691 | doi = 10.1042/BST0310228 }}
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• {{cite journal | vauthors = Cheung PC, Salt IP, Davies SP, Hardie DG, Carling D | title = Characterization of AMP-activated protein kinase gamma-subunit isoforms and their role in AMP binding | journal = The Biochemical Journal | volume = 346 Pt 3 | issue = Pt 3 | pages = 659–669 | date = March 2000 | pmid = 10698692 | pmc = 1220898 | doi = 10.1042/0264-6021:3460659 }}
• {{cite journal | vauthors = Lang T, Yu L, Tu Q, Jiang J, Chen Z, Xin Y, Liu G, Zhao S | title = Molecular cloning, genomic organization, and mapping of PRKAG2, a heart abundant gamma2 subunit of 5'-AMP-activated protein kinase, to human chromosome 7q36 | journal = Genomics | volume = 70 | issue = 2 | pages = 258–263 | date = December 2000 | pmid = 11112354 | doi = 10.1006/geno.2000.6376 }}
• {{cite journal | vauthors = Blair E, Redwood C, Ashrafian H, Oliveira M, Broxholme J, Kerr B, Salmon A, Ostman-Smith I, Watkins H | title = Mutations in the gamma(2) subunit of AMP-activated protein kinase cause familial hypertrophic cardiomyopathy: evidence for the central role of energy compromise in disease pathogenesis | journal = Human Molecular Genetics | volume = 10 | issue = 11 | pages = 1215–1220 | date = May 2001 | pmid = 11371514 | doi = 10.1093/hmg/10.11.1215 | doi-access = free }}
• {{cite journal | vauthors = Gollob MH, Green MS, Tang AS, Gollob T, Karibe A, Ali Hassan AS, Ahmad F, Lozado R, Shah G, Fananapazir L, Bachinski LL, Roberts R | title = Identification of a gene responsible for familial Wolff-Parkinson-White syndrome | journal = The New England Journal of Medicine | volume = 344 | issue = 24 | pages = 1823–1831 | date = June 2001 | pmid = 11407343 | doi = 10.1056/NEJM200106143442403 | doi-access = free }}
• {{cite journal | vauthors = Hamilton SR, Stapleton D, O'Donnell JB, Kung JT, Dalal SR, Kemp BE, Witters LA | title = An activating mutation in the gamma1 subunit of the AMP-activated protein kinase | journal = FEBS Letters | volume = 500 | issue = 3 | pages = 163–168 | date = July 2001 | pmid = 11445078 | doi = 10.1016/S0014-5793(01)02602-3 | s2cid = 85248755 | doi-access = free | bibcode = 2001FEBSL.500..163H }}

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• [https://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=hyper-card GeneReviews/NCBI/NIH/UW entry on Familial Hypertrophic Cardiomyopathy Overview]

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Category:EC 2.7.11

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