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. Author manuscript; available in PMC: 2009 Mar 9.
Published in final edited form as: Am J Med Genet C Semin Med Genet. 2006 May 15;142C(2):95–103. doi: 10.1002/ajmg.c.30089

Figure 1.

Figure 1

The catabolic pathway of leucine. Isovaleryl-CoA dehydrogenase catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA. Enzyme deficiency results in the accumulation of isovaleryl-CoA derivatives.