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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

References

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