Isobutyryl coenzyme A (isobutyryl-CoA) is a short-chain branched acyl CoA. Isobutyryl-CoA is a substrate for isobutyryl-CoA dehydrogenase (IBDH) in the catabolism of valine and an intermediate in the synthesis of isobutyryl-L-carnitine , which accumulates in IBDH deficiency.
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