Journal of Inherited Metabolic Disease 2012-01-01

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

Ronald J A Wanders, Marinus Duran, Ference J Loupatty

Index: J. Inherit. Metab. Dis. 35 , 5-12, (2012)

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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.