pyruvate dehydrogenase

Function

• catalyzes conversion of pyruvate to acetyl-CoA & CO2

Structure

• contains multiple copies of three enzymatic components:
• pyruvate dehydrogenase (E1)
• dihydrolipoamide acetyltransferase (E2)
• 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

More general terms

• multienzyme complex
• ketone oxidoreductase
• phosphoprotein
• dehydrogenase

Additional terms

• pyruvate dehydrogenase deficiency

Database

• OMIM: https://mirror.omim.org/entry/246900