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  • 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
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.

MATERIALS
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Product Description

Sigma-Aldrich
(±)-Sodium β-hydroxyisobutyrate, ≥96.0%
Sigma-Aldrich
Sodium (R)-β-hydroxyisobutyrate, ≥96.0%
Sigma-Aldrich
β-Hydroxybutyrate Dehydrogenase from Pseudomonas lemoignei, lyophilized powder, ≥200 units/mg protein
Sigma-Aldrich
Sodium (S)-β-hydroxyisobutyrate, ≥96.0%
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β-Hydroxybutyrate Dehydrogenase from Rhodopseudomonas sphaeroides, Type V, lyophilized powder, 250-750 units/mg protein