PDHA1 The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle. Ubiquitous. 4 alternatively spliced human isoforms have been reported. 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 1.2.4.1; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: Xp22.12
Cellular Component:  mitochondrial matrix; mitochondrion; nucleolus; pyruvate dehydrogenase complex
Molecular Function:  protein binding; pyruvate dehydrogenase (acetyl-transferring) activity; pyruvate dehydrogenase (NAD+) activity; pyruvate dehydrogenase activity
Biological Process:  acetyl-CoA biosynthetic process from pyruvate; glucose metabolic process; mitochondrial acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process; tricarboxylic acid cycle
Disease: Pyruvate Dehydrogenase E1-alpha Deficiency
Reference #:  P08559 (UniProtKB)
Alt. Names/Synonyms: ODPA; PDHA; PDHA1; PDHAD; PDHCE1A; PDHE1-A type I; PHE1A; pyruvate dehydrogenase (lipoamide) alpha 1; pyruvate dehydrogenase alpha 1; pyruvate dehydrogenase complex, E1-alpha polypeptide 1; pyruvate dehydrogenase E1 alpha 1; pyruvate dehydrogenase E1 alpha 1 subunit; Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; pyruvate dehydrogenase E1 subunit alpha 1
Gene Symbols: PDHA1
Molecular weight: 43,296 Da
Basal Isoelectric point: 8.35  Predict pI for various phosphorylation states
CST Pathways:  Warburg Effect
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
Select Structure to View Below

PDHA1

Protein Structure Not Found.


Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene