PDP1,2 dephosphorylate p-lipo-PDH

Stable Identifier
Reaction [transition]
Homo sapiens
PDP-catalyzed dephosphorylation (activation) of phospho E1 alpha subunit, Activation of PDC by dephosphorylation of phospho-E1 alpha component
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Pyruvate dehydrogenase phosphatase (PDP) in the mitochondrial matrix catalyzes the hydrolytic removal of phosphate groups from phosphoserine residues in the E1 alpha subunit of the pyruvate dehydrogenase complex. The active form of PDP is a heterodimer of a catalytic subunit and a regulatory one. Two isoforms of the catalytic subunit have been identified and biochemically characterized (Huang et al. 1998) and mutations in PDP1 have been associated with PDP deficiency in vivo (Maj et al. 2005). The properties of the human regulatory subunit have been deduced from those of its bovine homologue (Lawson et al. 1997). The activity of PDP1 is greatly enhanced through Ca2+ -dependent binding of the catalytic subunit (PDP1c) to the L2 (inner lipoyl) domain of dihydrolipoyl acetyltransferase (E2), which is also integrated in the pyruvate dehydrogenase complex. PDP activity requires Mg2+ (Huang et al. 1998).
Literature References
PubMed ID Title Journal Year
9651365 Isoenzymes of pyruvate dehydrogenase phosphatase. DNA-derived amino acid sequences, expression, and regulation

Harris, RA, Gudi, R, Huang, B, Popov, KM, Wu, P, Hamilton, J

J Biol Chem 1998
9395502 Cloning, expression, and properties of the regulatory subunit of bovine pyruvate dehydrogenase phosphatase

Park, SH, Mattison, AR, Yan, J, Reed, LJ, Lawson, JE

J Biol Chem 1997
16574315 Pyruvate dehydrogenase phosphatase deficiency: orphan disease or an under-diagnosed condition?

Cameron, JM, Robinson, BH, Maj, MC

Mol Cell Endocrinol 2006
15855260 Pyruvate dehydrogenase phosphatase deficiency: identification of the first mutation in two brothers and restoration of activity by protein complementation

Baumgartner, ER, Maj, MC, Robinson, BH, Cameron, JM, Baumgartner, MR, MacKay, N, Addis, J, Levandovskiy, V

J Clin Endocrinol Metab 2005
17532339 Crystal structure of pyruvate dehydrogenase phosphatase 1 and its functional implications

Vassylyev, DG, Symersky, J

J Mol Biol 2007
Catalyst Activity

[pyruvate dehydrogenase (lipoamide)] phosphatase activity of PDP [mitochondrial matrix]

This event is regulated
Orthologous Events
Cross References
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