a protein phosphoserine phosphatase associated with the mitochondrial matrix that activates phosphorylated pyruvate dehydrogenase complex by dephosphorylation. The PDPs play crucial roles in switching metabolic flux from glycolysis towards oxidative phosphorylation. Catalyzes the dephosphorylation and concomitant reactivation of the alpha subunit of the E1 component of the pyruvate dehydrogenase complex . PDP1 is composed of a catalytic subunit and a regulatory subunit (PDPR). Heterodimer of a catalytic subunit and a FAD protein of unknown function . Defects in PDP1 are the cause of pyruvate dehydrogenase phosphatase deficiency (PDP deficiency). PDP deficiency results in lactic acidosis leading to neurological dysfunction. Belongs to the PP2C family. Note: This description may include information from UniProtKB.
Protein type: EC 126.96.36.199; Mitochondrial; Motility/polarity/chemotaxis; Protein phosphatase, Ser/Thr (non-receptor)