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Disorders of propionate and methylmalonate metabolism

  • Chapter
Organic Acids in Man

Abstract

The final unique stage in the metabolism of L-isoleucine involves the cleavage of 2-methylacetoacetyl-CoA to acetyl-CoA and propionyl-CoA (Section 10.4). The propionyl-CoA is further metabolized to methylmalonyl-CoA by a biotin-dependent carboxylase and subsequently via succinyl-CoA into the tricarboxylic acid cycle. L-Valine is also metabolized ultimately to methylmalonyl-CoA (Section 10.4), and thus these two branched-chain amino acids form the major precursors of propionyl-CoA and methylmalonyl-CoA. Other precursors of propionyl-CoA include methionine, threonine, oddcarbon-number fatty acids and cholesterol. The methylmalonyl-CoA produced by propionyl-CoA carboxylase occurs as the D(S)-enantiomer and is racemized to the L(R)-enantiomer by methylmalonyl-CoA racemase. L(R)Methylmalonyl-CoA is then metabolized to succinyl-CoA by a vitamin B12-dependent mutase prior to introduction of the modified molecule into the tricarboxylic acid cycle.

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  1. M.R.C. Clinical Research Centre, Harrow, UK

    R. A. Chalmers & A. M. Lawson

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© 1982 R. A. Chalmers and A. M. Lawson

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Chalmers, R.A., Lawson, A.M. (1982). Disorders of propionate and methylmalonate metabolism. In: Organic Acids in Man. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-5778-7_11

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  • DOI: https://doi.org/10.1007/978-94-009-5778-7_11

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