試題詳解

77. A deficiency of branched chain α-keto acid dehydrogenease complex causes the defect in metabolism of amino acids. The accumulation of α-keto acid in urine leads to maple syrup urine disease. Which of the following amino acids are metabolized by α-keto acid dehydrogenease complex?            1.Methionine2.Leucine3.Isoleucine4.Valine5.Alanine
(A)125
(B)135
(C)234
(D)245
(E)345