You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information.
What is Isovaleric academia is caused by deficiency of - and explain in detail?
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.