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Protein Page:
PDHB (human)

PDHB the beta subunit of pyruvate dehydrogenase (PDH), a 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. Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency), the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS). Two alternatively spliced human isoforms have been described. 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: 3p21.1-p14.2
Cellular Component: mitochondrial matrix; mitochondrion; nucleoplasm; nucleus; pyruvate dehydrogenase complex
Molecular Function: protein binding; pyruvate dehydrogenase activity
Biological Process: acetyl-CoA biosynthetic process from pyruvate; glyoxylate metabolic process; pyruvate metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; tricarboxylic acid cycle
Disease: Pyruvate Dehydrogenase E1-beta Deficiency
Reference #:  P11177 (UniProtKB)
Alt. Names/Synonyms: DKFZp564K0164; ODPB; PDHB; PDHE1-B; PHE1B; pyruvate dehydrogenase (lipoamide) beta; Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; pyruvate dehydrogenase, E1 beta polypeptide
Gene Symbols: PDHB
Molecular weight: 39,233 Da
Basal Isoelectric point: 6.2  Predict pI for various phosphorylation states
CST Pathways:  Warburg Effect
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