PDP1,2 dephosphorylate p-lipo-PDH
2 H2O + p-lipo-PDH → PDH complex + 2 Pi
- Reactome
- R-HSA-204169
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:12670
Inputs
- 2 H2OCHEBI:15377, H2O, mitochondrial matrix
- p-lipo-PDHR-HSA-210342, mitochondrial matrix
Outputs
- PDH complexR-HSA-70070, mitochondrial matrix
- 2 PiCHEBI:43474, HO4P, mitochondrial matrix
Catalysed by
- [pyruvate dehydrogenase (acetyl-transferring)]-phosphatase activity of PDP [mitochondrial matrix]EC 3.1.3.43
Literature
- Isoenzymes of pyruvate dehydrogenase phosphatase. DNA-derived amino acid sequences, expression, and regulation J Biol Chem, 1998
- Cloning, expression, and properties of the regulatory subunit of bovine pyruvate dehydrogenase phosphatase J Biol Chem, 1997
- Pyruvate dehydrogenase phosphatase deficiency: identification of the first mutation in two brothers and restoration of activity by protein complementation J Clin Endocrinol Metab, 2005
- Crystal structure of pyruvate dehydrogenase phosphatase 1 and its functional implications J Mol Biol, 2007
- Pyruvate dehydrogenase phosphatase deficiency: orphan disease or an under-diagnosed condition? Mol Cell Endocrinol, 2006