Sweaty feet odour in body fluids occur due to deficiency of: (A) Biotin, (B) Isovaleryl-CoA dehydrogenase, (C) Thiamine linked decarboxylase, (D) Leucine transaminase
Answer is B. The impaired enzyme in isovaleric acidemia is isovaleryl-CoA dehydrogenase. Vomiting, acidosis and coma follow ingestion of excess protein. Accumulated isovaleryl-CoA is hydrolyzed to isovalerate and excreted. Characteristic odor of sweaty feet is present.
Harper's Illustrated Biochemistry 30 th edition page no 309