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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

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2007
2007
INTRODUCTION Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder caused by an inherited deficiency of… 
Highly Cited
2001
Highly Cited
2001
Untreated maple syrup urine disease (MSUD) results in mental and physical disabilities and often leads to neonatal death. Newborn… 
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… 
1998
1998
Mutation analysis of DNA from cultured amniocytes with absent branched‐chain α‐ketoacid dehydrogenase activity revealed a C to T… 
1998
1998
A novel single base pair deletion in the acid sphingomyelinase (ASM) gene (677delT in the cDNA) was identified in 12 Israeli Arab… 
1993
1993
Maple syrup urine disease results from inherited defects in human nuclear genes for branched chain alpha-ketoacid dehydrogenase… 
1985
1985
A radiochemical assay was developed for measuring branched-chain alpha-ketoacid dehydrogenase activity of Triton X-100 extracts…