Why does ketogenic amino acids help pyruvate dehydrogenase deficiency? - USMLE Forums
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Old 07-07-2011
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Biochemistry Why does ketogenic amino acids help pyruvate dehydrogenase deficiency?

First Aid says pyruvate dehydrogenase deficiency can be helped by taking food containing ketogentic amino acids (leucine and lysine), can someone explain why?

Thanks so much
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Old 07-07-2011
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In pyruvate dehydrogenase complex deficiency there will be three enzyme deficient in carbohydrate metabolism results in mental retardation, other neurologic features, lactic acidosis, wide variety of clinical features.
Amino acids like lysine and leucine can be safely administered to pyruvate dehydrogenase deficient patients because these amino acids are ketogenic and would not increase blood lactate levels
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Old 07-07-2011
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Thank you, Confident. Your explanation is very clear and right to the point!

Really appreciated! All the best..........
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Old 07-08-2011
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Quote:
Originally Posted by swiftdeck View Post
I thought it was because pyruvate dehydrogenase makes Acetyl-CoA, and as it's deficient, requires another means of gaining Acetyl-CoA. Ketogenic amino acids are able to be converted to Acetyl-CoA, and so help make ATP.
Also patient will be having lactic acidosis so it should be substituted with amino acid which does not raise lactic acid level as well.
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Old 07-18-2011
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Quote:
Originally Posted by swiftdeck View Post
I thought it was because pyruvate dehydrogenase makes Acetyl-CoA, and as it's deficient, requires another means of gaining Acetyl-CoA. Ketogenic amino acids are able to be converted to Acetyl-CoA, and so help make ATP.
Both sound foot to me.
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Old 08-26-2011
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in PD deficiency the fellow cannot shunt the pyruvate made during glycolysis into the Krebs cycle, so glucose and glucogenic amino acids are going to compound his problems. ketogenic aa however will cause production of ketones. now these ketones can be used directly as a source of energy eg beta hydroxybutyrate in the brain or they can be converted to acetylCoA in the liver and thus enter kreb cycle, so by giving ketogenic amino acids you have bypassed the problem of pyruvate being unable to be converted to acetylCoA
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