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

Journal of Inherited Metabolic Disease (2012) 35(1) 5-12
DOI: 10.1007/s10545-010-9236-x
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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. © The Author(s) 2010.

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Wanders, R. J. A., Duran, M., & Loupatty, F. J. (2012). Enzymology of the branched-chain amino acid oxidation disorders: The valine pathway. Journal of Inherited Metabolic Disease, 35(1), 5–12. https://doi.org/10.1007/s10545-010-9236-x

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