ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease
J. Clin. Invest. 113:3 doi:10.1172/JCI19574
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Figure 1

Schematic catabolic pathway of branched-chain amino acids valine (Val), leucine (Leu), and isoleucine (Ile). MCF, mitochondrial carrier family.