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Merck

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
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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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Sigma-Aldrich
Sodium (R)-ฮฒ-hydroxyisobutyrate, โ‰ฅ96.0%
Sigma-Aldrich
(ยฑ)-Sodium ฮฒ-hydroxyisobutyrate, ≥96.0%
Sigma-Aldrich
ฮฒ-Hydroxybutyrate Dehydrogenase from Pseudomonas lemoignei, lyophilized powder, โ‰ฅ200 units/mg protein
Sigma-Aldrich
ฮฒ-Hydroxybutyrate Dehydrogenase from Rhodopseudomonas sphaeroides, Type V, lyophilized powder, 250-750 units/mg protein
Sigma-Aldrich
Sodium (S)-ฮฒ-hydroxyisobutyrate, โ‰ฅ96.0%