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Valine catabolic pathway showing formation of metabolites listed in Table 4, which are abnormal in SCEH and HIBCH deficiency. The metabolic origin of 2,3‐dihydroxy‐2‐methylbutyrate is currently unclear. Enzymes are numbered: 2 = propionyl‐CoA carboxylase; 3 = (R)‐methylmalonyl‐CoA mutase; 5 = isobutyryl CoA dehydrogenase. [Color figure can be viewed at http://wileyonlinelibrary.com]
