4.4 Article Proceedings Paper

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

Journal

JOURNAL OF INHERITED METABOLIC DISEASE
Volume 35, Issue 1, Pages 5-12

Publisher

WILEY
DOI: 10.1007/s10545-010-9236-x

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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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