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Enzymology of the branched‐chain amino acid oxidation disorders: the valine pathway
Author(s) -
Wanders Ronald J. A.,
Duran Marinus,
Loupatty Ference J.
Publication year - 2012
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-010-9236-x
Subject(s) - valine , amino acid , biochemistry , mitochondrion , branched chain amino acid , maple syrup urine disease , chemistry , oxidation reduction , stereochemistry , biology , leucine
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.