The Citric Acid Cycle Flashcards
What is necessary in the cell for the citric acid cycle to occur?
Mitochondria
What is the function of coenzyme A?
Coenzyme A (A for acetylation) was found as a cofactor for the acetylation reactions and is needed for pyruvate oxidation and to participate in citric acid synthesis
What does the oxidation of AcCoA produce?
3 NADH
1 FADH2
1 GTP
Where are the TCA cycle enzymes found?
Mitochondrial Matrix
Where does the ATP in the TCA cycle come from?
The transfer of electrons from substrates to molecular oxygen provides the energy to synthesize ATP
What TCA enzymes are controlled by allosteric regulation?
Isocitrate Dehydrogenase
α-ketoglutarate Dehydrogenase
What will increased ADP do to the TCA cycle?
The CAC (citric acid cycle) activity will be increased
What happens with a decreased supply of acetyl-CoA?
Pyruvate dehydrogenase activity will increase to convert glucose to AcCoA.
Fatty acid oxidation into AcCoA will increase.
What is the most common cause of pyruvate dehydrogenase deficiency?
The most common form of pyruvate dehydrogenase deficiency is caused by mutations in the E1 alpha gene, and is inherited in an X-linked dominant manner.
All other forms are caused by various genes and are inherited in an autlosomal recessive manner.
What are the symptoms of pyruvate dehydrogenase deficiency?
Children with pyruvate dehydrogenase deficiency exhibit elevated serum levels of lactate, pyruvate and alanine, which produce a chronic lactic acidosis.
What are the symptoms of fumarase deficiency?
- Severe neurological impairment
- Increased urinary excretion of fumarate, succinate, alpha keto-glutarate and citrate
