- Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway.
Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway.
Journal of inherited metabolic disease (2010-11-26)
Ronald J A Wanders, Marinus Duran, Ference J Loupatty
PMID21104317
RÉSUMÉ
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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β-Hydroxybutyrate Dehydrogenase from Pseudomonas lemoignei, lyophilized powder, ≥200 units/mg protein
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β-Hydroxybutyrate Dehydrogenase from Rhodopseudomonas sphaeroides, Type V, lyophilized powder, 250-750 units/mg protein