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

Product Number

Brand

Product Description

H8509

Sigma-Aldrich

β-Hydroxybutyrate Dehydrogenase from Rhodopseudomonas sphaeroides, Type V, lyophilized powder, 250-750 units/mg protein

11161

Sigma-Aldrich

Sodium (R)-β-hydroxyisobutyrate, ≥96.0%

36105

Sigma-Aldrich

(±)-Sodium β-hydroxyisobutyrate, ≥96.0%

H9408

Sigma-Aldrich

β-Hydroxybutyrate Dehydrogenase from Pseudomonas lemoignei, lyophilized powder, ≥200 units/mg protein