OXIDATION OF PYRUVATE
- What are the three enzymes of the pyruvate dehydrogenase (PDH) complex?
- Pyruvate decarboxylase
- Dihydrolipoyl transacetylase
- dihydrolipoyl dehydrogenase
- What reaction does the PDH complex catalyze?
- Pyruvate + NAD+ + CoA → acetyl CoA CO2 + NADH
- What coenzymes are required by this enzyme?
- 1.Thiamine pyrophosphate (vitamin B1)
- 2.coenzyme A (vitamin B5),
- 3.NAD+ (vitamin B3),
- 4.flavin adenine dinucleotide (FAD) (vitamin B2),
- 5. lipoic acid
- The PDH complex is similar to what other enzyme?
- α-Ketoglutarate dehydrogenase complex
- Is the PDH complex active in the phosphorylated or nonphosphorylated state?
- Nonphosphorylated state
- What enzyme phosphorylates the PDH complex?
- PDH kinase
- What molecules activate PDH kinase (thus inhibiting the PDH complex)?
- Acetyl CoA, NADH
- What molecules inhibit PDH kinase (thus activating the pyruvate dehydrogenase complex)?
- Pyruvate, decreased levels of adenosine diphosphate (ADP)
- What enzyme dephosphorylates the PDH complex?
- PDH phosphorylase (PDH phosphatase)
- What molecule activates PDH phosphatase (thus activating the PDH complex)?
- Ca2+
- What are the major manifestations of PDH deficiency?
- Lactic acidosis, neurological manifestations
- What is the treatment for PDH deficiency?
- Increased intake of ketogenic nutrients
