A baby is hypotonic and shows increased ratio of Pyruvate to Acetyl CoA. Pyruvate cannot form Acetyl CoA in fibroblast. He also shows features of lactic acidosis. Which of the following can revert the situation?
Ans. D. ThiaminIncreased Pyruvate to Acetyl-CoA, with lactic acidosis, inability to convert Pyruvate to Acetyl-CoA are all suggestive of a Pyruvate Dehydrogenase deficiency. Thiamine Pyrophosphate is one of the coenzymes of PDH. Hence Thiamine is the answer.
Biotin
Pyridoxine
Free fatty acid
Thiamin
Carbohydrates