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Biochemistry
A 2-week-old neonate with complete hypotonia, convulsions, failure to thrive and metabolic acidosis. The baby has small of burnt sugar in urine. The test called DNPH test is positive. What is the enzyme deficiency in this metabolic disorder?
Isovaleryl CoA dehydrogenase
Dihydrolipoamide dehydrogenase
Branched chain keto acid dehydrogenase
Transacylase
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