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PDP1 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; Mitochondrial; Motility/polarity/chemotaxis; Protein phosphatase, Ser/Thr (non-receptor)
Chromosomal Location of Human Ortholog: 8q22.1
Cellular Component: mitochondrial matrix
Molecular Function: [pyruvate dehydrogenase (lipoamide)] phosphatase activity; metal ion binding; protein binding; protein serine/threonine phosphatase activity
Biological Process: regulation of acetyl-CoA biosynthetic process from pyruvate
Disease: Pyruvate Dehydrogenase Phosphatase Deficiency
Reference #:  Q9P0J1 (UniProtKB)
Alt. Names/Synonyms: [Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial; FLJ32517; FLJ56179; MGC119646; PDH; PDP; PDP 1; PDP1; PDPC; PDPC 1; PPM2C; Protein phosphatase 2C; protein phosphatase 2C, magnesium-dependent, catalytic subunit; pyruvate dehydrogenase (Lipoamide) phosphatase-phosphatase; Pyruvate dehydrogenase phosphatase catalytic subunit 1; pyruvate dehyrogenase phosphatase catalytic subunit 1
Gene Symbols: PDP1
Molecular weight: 61,054 Da
Basal Isoelectric point: 6.2  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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