Essential Biochemistry - Protein Metabolism (Section 3 - No. 34)
In Maple-syrup urine disease, which of the following compounds is accumulated?
Homogentisate
Methylmalonyl-CoA
Branched chain α-keto acids
Homocystine
Explanation
Branched-chain α-keto acids accumulate in Maple-syrup urine disease.
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