A baby is hypotonic and shows that pyruvate cannot form Acetyl CoA in fibroblasts. Also lactic acidosis is found. Administration of which of the following can reve this situation:
Correct Answer: Thiamine
Description: Congenital Lactic Acidosis may occur due to defect in thiamine (B1) which is required for Pyruvate dehydrogenase (PDH) which conves pyruvate to Acetyl CoA. PDH requires 5 coenzymes: Lipoic acid and four B-complex Vitamins - Thiamine -B1; Riboflavin-B2 or FAD; Niacin-B3 or NAD; Pantothenic acid-B5). In case of Thiamine deficiency, this reaction will be affected and pyruvate will accumulate leading to the formation of lactate and finally leading to Lactic Acidosis (see fig).
Category:
Biochemistry
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