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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
INTRODUCTION Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder caused by an inherited deficiency of… Expand
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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… Expand
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Highly Cited
1998
Highly Cited
1998
The metabolism of valine to isobutyl alcohol in yeast was examined by 13C nuclear magnetic resonance spectroscopy and combined… Expand
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1998
1998
A novel single base pair deletion in the acid sphingomyelinase (ASM) gene (677delT in the cDNA) was identified in 12 Israeli Arab… Expand
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1998
1998
Mutation analysis of DNA from cultured amniocytes with absent branched-chain alpha-ketoacid dehydrogenase activity revealed a C… Expand
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1993
1993
Maple syrup urine disease results from inherited defects in human nuclear genes for branched chain alpha-ketoacid dehydrogenase… Expand
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1985
1985
A radiochemical assay was developed for measuring branched-chain alpha-ketoacid dehydrogenase activity of Triton X-100 extracts… Expand
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