pyruvate dehydrogenase

Function: - catalyzes conversion of pyruvate to acetyl-CoA & CO₂ Structure: - contains multiple copies of three enzymatic components: 1) pyruvate dehydrogenase (E1) 2) dihydrolipoamide acetyltransferase (E2) 3) lipoamide dehydrogenase (E3) - 20 to 30 alpha(2)-beta(2) tetramers of E1 + 6 homodimers of E3 + 60 copies of E2 Compartment: mitochondrial matrix Pathology: - defects in components associated with pyruvate dehydrogenase deficiency Laboratory: - pyruvate dehydrogenase complex in fibroblasts - pyruvate dehydrogenase complex in tissue

General

dehydrogenase ketone oxidoreductase multienzyme complex phosphoprotein

Properties

COMPARTMENT: mitochondrial matrix MOTIF: active site phosphorylation site

References

UniProt :accession P10515

Components

dihydrolipamide acetyltransferase E2 (pyruvate dehydrogenase E2, DLAT) lipoamide dehydrogenase (glycine cleavage system L protein, DLD, GCSL, LAD, LADH, PHE3) pyruvate dehydrogenase E1