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The significance of this reaction is that the level of 2MBC increases in the context of defects in isoleucine catabolism, particularly isoleucine degradation, particularly short/branched-chain acyl-CoA dehydrogenase (SBCAD) deficiency

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Cammi, R

Energetics of 3-oxo-.DELTA.5-steroid Isomerase: Source of the Catalytic Power of the Enzyme