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Pyruvate dehydrogenase complex – steps, regulation, and inhibitors
Pyruvate dehydrogenase complex deficiency results in impaired conversion of pyruvate to acetyl-CoA, reduced production of citrate, and thus an impaired TCA cycle, leading to severe energy deficits (especially in the CNS). Long-term treatment includes a ketogenic diet (high fat, low carbohydrate, glucogenic amino acids, e.g. valine) and cofactor supplementation with thiamine and lipoic acid.
The five cofactors of the pyruvate dehydrogenase complex: Tender (Thiamine) Loving (lipoic acid) Care (CoA) For (FAD) Nancy (NAD+).
Arsenic inhibits lipoic acid, thereby preventing the production of acetyl-CoA and inhibiting the TCA cycle!
www.medvizz.com
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