| PDHA1 Protein — Pyruvate Dehydrogenase E1 Alpha 1 | |
|---|---|
| Gene | [PDHA1](/genes/pdha1) |
| UniProt | P21987 |
| PDB | 3EXE |
| Mol. Weight | 43 kDa |
| Localization | Mitochondrial matrix |
| Family | Pyruvate dehydrogenase complex (PDC) family |
| Diseases | [Alzheimer's Disease](/diseases/alzheimers), [Parkinson's Disease](/diseases/parkinsons-disease), [PDH Deficiency](/diseases/neurons) |
PDHA1 encodes the E1 alpha subunit of the pyruvate dehydrogenase complex (PDC), a key enzyme complex that links glycolysis to the citric acid cycle by converting pyruvate to acetyl-CoA[1]. The PDH complex is a rate-limiting step in glucose metabolism, and its dysfunction is strongly implicated in neurodegenerative diseases including Alzheimer's Disease and Parkinson's Disease[2].
PDHA1 is one of two genes encoding the E1 alpha subunit (PDHA1 and PDHA2). PDHA1 is expressed in most tissues, while PDHA2 is testis-specific. The E1 complex is a heterotetramer of two alpha and two beta subunits[3].
The pyruvate dehydrogenase complex consists of:
PDHA1 features:
Nature reviews. Mitochondrial DNA repair and neurodegeneration. Nature Reviews Neuroscience. 2010. ↩︎
DiMauro S, Schon EA. Mitochondrial respiratory-chain diseases. New England Journal of Medicine. 2003. ↩︎
Lin MT, Beal MF. Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature. 2006. ↩︎