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alpha-ketoacid dehydrogenase activity

Catalysis of an oxidation-reduction (redox) reaction involving an alpha-ketoacid. [GOC:mah]
National Institutes of Health

Papers overview

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2007
2007
[Advances in the diagnosis and treatment of maple syrup urine disease: experience in Galicia (Spain)].
INTRODUCTION Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder caused by an inherited deficiency of… 
Highly Cited
1998
Highly Cited
1998
An Investigation of the Metabolism of Valine to Isobutyl Alcohol in Saccharomyces cerevisiae *
The metabolism of valine to isobutyl alcohol in yeast was examined by 13C nuclear magnetic resonance spectroscopy and combined… 
1998
1998
A nonsense mutation (R242X) in the branched-chain alpha-keto acid dehydrogenase E1alpha subunit gene (BCKDHA) as a cause of maple syrup urine disease. Mutations in brief no. 160. Online.
Mutation analysis of DNA from cultured amniocytes with absent branched-chain alpha-ketoacid dehydrogenase activity revealed a C… 
1993
1993
Thiamin-responsive maple syrup urine disease in a patient antigenically missing dihydrolipoamide acyltransferase.
Maple syrup urine disease results from inherited defects in human nuclear genes for branched chain alpha-ketoacid dehydrogenase… 
1985
1985
Physiological covalent regulation of rat liver branched-chain alpha-ketoacid dehydrogenase.
A radiochemical assay was developed for measuring branched-chain alpha-ketoacid dehydrogenase activity of Triton X-100 extracts… 
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