Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway

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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Bibliographic Details
Published in:Journal of inherited metabolic disease 2012-01, Vol.35 (1), p.5-12
Main Authors: Wanders, Ronald J. A., Duran, Marinus, Loupatty, Ference J.
Format: Article
Language:English
Subjects:
Amino Acid Metabolism, Inborn Errors - diagnosis
Amino Acid Metabolism, Inborn Errors - metabolism
Amino Acids, Branched-Chain - metabolism
Animals
Biochemistry
Biological and medical sciences
Branched-Chain Amino Acids
Enoyl-CoA Hydratase - metabolism
Human Genetics
Humans
Hydroxybutyrate Dehydrogenase - metabolism
Internal Medicine
Medical genetics
Medical sciences
Medicine
Medicine & Public Health
Metabolic Diseases
Methylmalonate-Semialdehyde Dehydrogenase (Acylating) - metabolism
Mitochondria - metabolism
Models, Chemical
Oxidoreductases Acting on CH-CH Group Donors - metabolism
Oxygen - chemistry
Pediatrics
Thiolester Hydrolases - metabolism
Valine - chemistry
Valine - genetics
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Description
Summary: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.
ISSN:0141-8955
1573-2665
DOI:10.1007/s10545-010-9236-x