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mds25538-sup-0001-suppfig1.eps694KSupplementary Figure 1. Isoleucine and ketone body metabolism. Mitochondrial acetoacetyl-coenzyme A (CoA) thiolase (beta-ketothiolase [T2]) is implicated in the metabolism of ketone bodies and of the amino acid isoleucine. (Left) Ketone body use in extrahepatic tissues is illustrated. (Right) T2 catalyzes the final reaction of isoleucine degradation. The preceding enzyme, 2-methylhydroxybutyryl-CoA dehydrogenase (MHBD), is shown. The metabolites present in T2 and MHBD deficiencies (boxes) are indicated to the right of each enzyme. SCOT indicates 3-ketoacid CoA transferase.
mds25538-sup-0002-suppvideo1.MP474577KSupplementary Information Video

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