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PDHA2 a testis-specific mitochondrial matrix enzyme that catalyzes the oxidative decarboxylation of pyruvate, producing acetyl-CoA and CO2. A key enzyme in controlling the balance between lipid and glucose oxidation depending on substrate availability. The pyruvate dehydrogenase (PDH) holoenzyme is a multi-enzyme complex (PDHC) that contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. The activity of PDH is tightly regulated by phosphorylation. The phosphorylation of at least one of three specific serine residues in E1 subunit by PDHK inactivates the PDHC, while dephosphorylation by PDP restores its activity. Expressed in postmeiotic spermatogenic cells. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine biosynthesis; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - pyruvate; EC; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 4q22.3
Cellular Component: mitochondrial matrix; mitochondrion; nucleolus; nucleus
Molecular Function: pyruvate dehydrogenase (acetyl-transferring) activity
Biological Process: pyruvate metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate
Reference #:  P29803 (UniProtKB)
Alt. Names/Synonyms: MGC149517; MGC149518; ODPAT; PDHA2; PDHAL; PDHE1-A type II; pyruvate dehydrogenase (lipoamide) alpha 2; pyruvate dehydrogenase E1 alpha 2; Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial; pyruvate dehydrogenase, E1-alpha polypeptide, testis specific
Gene Symbols: PDHA2
Molecular weight: 42,933 Da
Basal Isoelectric point: 8.76  Predict pI for various phosphorylation states
CST Pathways:  Warburg Effect
Select Structure to View Below


Protein Structure Not Found.

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