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:
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).
Biotin
Pyridoxal phosphate
Thiamine
Pyruvate
Vitamins and Minerals