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Open Access 01-01-2012 | Branched-Chain Amino Acids

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

Authors: Ronald J. A. Wanders, Marinus Duran, Ference J. Loupatty

Published in: Journal of Inherited Metabolic Disease | Issue 1/2012

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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Title: Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway
Authors: Ronald J. A. Wanders
Marinus Duran
Ference J. Loupatty
Publication date: 01-01-2012
Publisher: Springer Netherlands
Published in: Journal of Inherited Metabolic Disease / Issue 1/2012
Print ISSN: 0141-8955
Electronic ISSN: 1573-2665
DOI: https://doi.org/10.1007/s10545-010-9236-x

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