alpha-ketoacid dehydrogenase activity

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

Topic mentions per year

Topic mentions per year

1985-2007
012319852007

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2007
2007
INTRODUCTION Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder caused by an inherited deficiency of… (More)
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2001
2001
Untreated maple syrup urine disease (MSUD) results in mental and physical disabilities and often leads to neonatal death. Newborn… (More)
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1998
1998
In adrenalectomized rats, acidosis does not increase whole-body leucine oxidation unless a physiologic amount of glucocorticoids… (More)
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1998
1998
The metabolism of valine to isobutyl alcohol in yeast was examined by 13C nuclear magnetic resonance spectroscopy and combined… (More)
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1998
1998
Mutation analysis of DNA from cultured amniocytes with absent branched-chain alpha-ketoacid dehydrogenase activity revealed a C… (More)
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1993
1993
Maple syrup urine disease results from inherited defects in human nuclear genes for branched chain alpha-ketoacid dehydrogenase… (More)
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1989
1989
Branched chain alpha-ketoacid dehydrogenase is a heteroprotein complex of mitochondria and commits the branched chain alpha… (More)
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1985
1985
A radiochemical assay was developed for measuring branched-chain alpha-ketoacid dehydrogenase activity of Triton X-100 extracts… (More)
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1985
1985
Branched chain amino acid (BCAA) transaminase activity increased in both the mitochondrial and supernatant fractions of brain… (More)
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