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The carbon chains of methionine, isoleucine, threonine, and valine are broken down to produce succinyl-CoA, which feeds into the Citric Acid Cycle. Methionine donates its methyl group via S-adenosylmethionine to one of several acceptors and the four remaining carbons are the backbone of propionyl-CoA that is subsequently converted to succinyl-CoA. The transdeamination and decarboxylation of valine are followed by a series of steps resulting in the formation of propionyl-CoA. Isoleucine also undergoes transdeamination, followed by oxidative decarboxylation. These steps yield propionyl-CoA as well as acetyl-CoA. Deficiency in the steps that convert isoleucine to propionyl-CoA causes sudden unexplained infant death, Reye-like syndrome, cardiomyopathy, and skeletal myopathy. (This definition may be outdated - see the DesignNote.)
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