Isovaleric academia is caused by deficiency of -

Ans. is 'c' i.e., Isovaleryl CoA dehydrogenase* Isovaleric acidemia is an inborn error of metabolism caused by a defect of Isovaleryl CoA dehydrogenase* Isovaleryl CoA dehydrogenase is an enzyme involved in the metabolism of a branched chain aminoacid, leucine.* It is characterized by failure to thrive, feeding difficulties, seizures, vomiting that progresses to coma. It is characterized by sweaty feet odour.* Diagnosis is done by estimation of isovalerylglycine and isovaleryl carnitine. This is done by Tandem Mass Spectrometry* Treatment is by protein restriction, glycine and carnitine supplementation (Isovaleryl CoA is conjugated with glycine and carnitine to form Isovaleryl glycine and Isovaleryl Carnitine and get excreted), Biotin Supplementation.