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

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

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65 Citations (Scopus)

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
Original languageEnglish
Pages (from-to)5-12
JournalJournal of Inherited Metabolic Disease
Volume35
Issue number1
DOIs
Publication statusPublished - 2012

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