Pyruvate dehydrogenase (E1) is one of the three components (E1, E2, and E3) of the large pyruvate dehydrogenase complex. Pyruvate dehydrogenase kinases catalyze phosphorylation of serine residues of E1 to inactivate the E1 component and inhibit the complex. Pyruvate dehydrogenase phosphatases catalyze the dephosphorylation and activation of the E1 component to reverse the effects of pyruvate dehydrogenase kinases. Pyruvate dehydrogenase phosphatase is a heterodimer consisting of catalytic and regulatory subunits. Two catalytic subunits have been reported; one is predominantly expressed in skeletal muscle and another one is much more abundant in the liver. The catalytic subunit, encoded by this gene, is the former, and belongs to the protein phosphatase 2C (PP2C) superfamily. Along with the pyruvate dehydrogenase complex and pyruvate dehydrogenase kinases, this enzyme is located in the mitochondrial matrix.
^Lawson JE, Niu XD, Browning KS, Trong HL, Yan J, Reed LJ (Sep 1993). "Molecular cloning and expression of the catalytic subunit of bovine pyruvate dehydrogenase phosphatase and sequence similarity with protein phosphatase 2C". Biochemistry32 (35): 8987–93. doi:10.1021/bi00086a002. PMID8396421.
Piccinini M, Mostert M, Alberto G, Ramondetti C, Novi RF, Dalmasso P, Rinaudo MT (Apr 2005). "Down-regulation of pyruvate dehydrogenase phosphatase in obese subjects is a defect that signals insulin resistance". Obesity Research13 (4): 678–86. doi:10.1038/oby.2005.76. PMID15897476.
Auffray C, Behar G, Bois F, Bouchier C, Da Silva C, Devignes MD, Duprat S, Houlgatte R, Jumeau MN, Lamy B (Feb 1995). "[IMAGE: molecular integration of the analysis of the human genome and its expression]". Comptes Rendus De L'Académie Des Sciences. Série III, Sciences De La Vie318 (2): 263–72. PMID7757816.
Lejeune F, Li X, Maquat LE (Sep 2003). "Nonsense-mediated mRNA decay in mammalian cells involves decapping, deadenylating, and exonucleolytic activities". Molecular Cell12 (3): 675–87. doi:10.1016/S1097-2765(03)00349-6. PMID14527413.
Cameron JM, Maj M, Levandovskiy V, Barnett CP, Blaser S, Mackay N, Raiman J, Feigenbaum A, Schulze A, Robinson BH (Apr 2009). "Pyruvate dehydrogenase phosphatase 1 (PDP1) null mutation produces a lethal infantile phenotype". Human Genetics125 (3): 319–26. doi:10.1007/s00439-009-0629-6. PMID19184109.
Maj MC, MacKay N, Levandovskiy V, Addis J, Baumgartner ER, Baumgartner MR, Robinson BH, Cameron JM (Jul 2005). "Pyruvate dehydrogenase phosphatase deficiency: identification of the first mutation in two brothers and restoration of activity by protein complementation". The Journal of Clinical Endocrinology and Metabolism90 (7): 4101–7. doi:10.1210/jc.2005-0123. PMID15855260.
Sugden MC, Holness MJ (May 2003). "Recent advances in mechanisms regulating glucose oxidation at the level of the pyruvate dehydrogenase complex by PDKs". American Journal of Physiology. Endocrinology and Metabolism284 (5): E855–62. doi:10.1152/ajpendo.00526.2002. PMID12676647.
Korotchkina LG, Patel MS (Jun 1995). "Mutagenesis studies of the phosphorylation sites of recombinant human pyruvate dehydrogenase. Site-specific regulation". The Journal of Biological Chemistry270 (24): 14297–304. doi:10.1074/jbc.270.24.14297. PMID7782287.
Stellingwerff T, Spriet LL, Watt MJ, Kimber NE, Hargreaves M, Hawley JA, Burke LM (Feb 2006). "Decreased PDH activation and glycogenolysis during exercise following fat adaptation with carbohydrate restoration". American Journal of Physiology. Endocrinology and Metabolism290 (2): E380–8. doi:10.1152/ajpendo.00268.2005. PMID16188909.
Ito M, Kobashi H, Naito E, Saijo T, Takeda E, Huq AH, Kuroda Y (Jul 1992). "Decrease of pyruvate dehydrogenase phosphatase activity in patients with congenital lactic acidemia". Clinica Chimica Acta; International Journal of Clinical Chemistry209 (1-2): 1–7. doi:10.1016/0009-8981(92)90327-M. PMID1327585.
Adams MD, Kerlavage AR, Fleischmann RD, Fuldner RA, Bult CJ, Lee NH, Kirkness EF, Weinstock KG, Gocayne JD, White O (Sep 1995). "Initial assessment of human gene diversity and expression patterns based upon 83 million nucleotides of cDNA sequence". Nature377 (6547 Suppl): 3–174. PMID7566098.
Caruso M, Maitan MA, Bifulco G, Miele C, Vigliotta G, Oriente F, Formisano P, Beguinot F (Nov 2001). "Activation and mitochondrial translocation of protein kinase Cdelta are necessary for insulin stimulation of pyruvate dehydrogenase complex activity in muscle and liver cells". The Journal of Biological Chemistry276 (48): 45088–97. doi:10.1074/jbc.M105451200. PMID11577086.
Bonaldo MF, Lennon G, Soares MB (Sep 1996). "Normalization and subtraction: two approaches to facilitate gene discovery". Genome Research6 (9): 791–806. doi:10.1101/gr.6.9.791. PMID8889548.
Huang B, Gudi R, Wu P, Harris RA, Hamilton J, Popov KM (Jul 1998). "Isoenzymes of pyruvate dehydrogenase phosphatase. DNA-derived amino acid sequences, expression, and regulation". The Journal of Biological Chemistry273 (28): 17680–8. doi:10.1074/jbc.273.28.17680. PMID9651365.