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branched-chain-2-oxoacid decarboxylase activity

Known as: BCKA, branched-chain oxo acid decarboxylase activity, (3S)-3-methyl-2-oxopentanoate carboxy-lyase (2-methylbutanal-forming) 
Catalysis of the reaction: (S)-3-methyl-2-oxopentanoate + H(+) = 2-methylbutanal + CO(2). [EC:4.1.1.72, RHEA:21111]
National Institutes of Health

Papers overview

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Highly Cited
2009
Highly Cited
2009
This study was conducted using the piglet model to test the hypothesis that mucosal cells of the neonatal small intestine can… Expand
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Review
2006
Review
2006
The three branched-chain amino acids (BCAAs) are the most hydrophobic of the amino acids and play crucial roles in determining… Expand
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Review
2006
Review
2006
During renal failure, abnormalities of BCAA and branched-chain keto acid (BCKA) metabolism are due to both the lack of renal… Expand
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Highly Cited
2004
Highly Cited
2004
Maple syrup urine disease (MSUD) is associated with increased branched-chain amino acids (BCAA), their keto acids (BCKA), and… Expand
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Highly Cited
2003
Highly Cited
2003
Maple syrup urine disease (MSUD) is an inborn error of amino acid metabolism, which affects the brain tissue resulting in… Expand
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Highly Cited
2001
Highly Cited
2001
A novel hypothesis for the role of branched-chain amino acids (BCAA) in regulating levels of the major excitatory… Expand
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Highly Cited
2000
Highly Cited
2000
The present experiment was designed to elucidate the mechanism of the methionine-sparing effect of excess branched-chain amino… Expand
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Highly Cited
1998
Highly Cited
1998
Abstract: Because it is well known that excess branched‐chain amino acids (BCAAs) have a profound influence on neurological… Expand
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Review
1984
Review
1984
INTRODUCTION… Expand
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Highly Cited
1981
Highly Cited
1981
Branched-chain alpha-keto and amino acid (BCKA, BCAA) concentrations were measured in blood, plasma, and tissues of rats fed low… Expand
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