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. 2012 Jan;35(1):5-12.
doi: 10.1007/s10545-010-9236-x. Epub 2010 Nov 23.

Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway

Ronald J A Wanders  1 , Marinus Duran, Ference J Loupatty
Affiliations

Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway

Ronald J A Wanders et al. J Inherit Metab Dis. 2012 Jan.

Abstract

Valine is one of the three branched-chain amino acids which undergoes oxidation within mitochondria. In this paper, we describe the current state of knowledge with respect to the enzymology of the valine oxidation pathway and the different disorders affecting oxidation.

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Figures

Fig. 1
Fig. 1
Schematic representation of the valine oxidation pathway and the enzymes involved (see text)
See this image and copyright information in PMC

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References

    1. Andresen BS, Christensen E, Corydon TJ, Bross P, Pilgaard B, Wanders RJA, Ruiter JPN, Simonsen H, Winter V, Knudsen I, Schroeder LD, Gregersen N, Skovby F. Isolated 2-Methylbutyrylglycinuria Caused by Short/Branched-Chain Acyl- CoA Dehydrogenase Deficiency: Identification of a New Enzyme Defect, Resolution of Its Molecular Basis, and Evidence for Distinct Acyl-CoA Dehydrogenases in Isoleucine And Valine Metabolism. Am J Hum Genet. 2000;67:1095–1103. doi: 10.1086/303105. - DOI - PMC - PubMed
    1. Bachhawat BK, Coon MJ, Kupiecki FP, Nagle R, Robinson WG. Coenzyme A thiol esters of isobutyric, methacrylic, and beta-hydroxyisobutyric acids as intermediates in the enzymatic degradation of valine. J Biol Chem. 1957;224:1–11. - PubMed
    1. Bannerjee D, Sanders LE, Sokatch JR. Properties of purified methylmalonate semialdehyde dehydrogenase of Pseudomonas aeruginosa. J Biol Chem. 1970;245:1828–1835. - PubMed
    1. Battaile KP, Nguyen TV, Vockley J, Kim JJ. Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases. J Biol Chem. 2004;279:16526–16534. doi: 10.1074/jbc.M400034200. - DOI - PubMed
    1. Boulat O, Benador N, Girardin E, Bachmann C. 3-hydroxyisobutyric aciduria with a mild clinical course. J Inherit Metab Dis. 1995;18:204–206. doi: 10.1007/BF00711767. - DOI - PubMed

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