Deficiency of butyryl-CoA dehydrogenase

Known as: Short-Chain Acyl-Coenzyme A Dehydrogenase Deficiency, Butyryl-CoA Dehydrogenase Deficiency, Short-Chain Acyl-CoA Dehydrogenase Deficiency (SCAD) 
A rare autosomal recessive inherited disorder caused by mutations in the ACADSB gene. It is characterized by deficiency of the enzyme 2-methylbutyryl… (More)
National Institutes of Health

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Highly Cited
2012
Highly Cited
2012
BACKGROUND Spontaneous coronary artery dissection (SCAD) is an acute coronary event of uncertain origin. Clinical features and… (More)
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Highly Cited
2009
Highly Cited
2009
This paper studies the sparsistency and rates of convergence for estimating sparse covariance and precision matrices based on… (More)
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Highly Cited
2008
Highly Cited
2008
The rapidly evolving field of metabolomics aims at a comprehensive measurement of ideally all endogenous metabolites in a cell or… (More)
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Highly Cited
2007
Highly Cited
2007
A reduced capacity for mitochondrial fatty acid oxidation in skeletal muscle has been proposed as a major factor leading to the… (More)
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Highly Cited
2005
Highly Cited
2005
Thiazolidenediones such as pioglitazone improve insulin sensitivity in diabetic patients by several mechanisms, including… (More)
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Highly Cited
2001
Highly Cited
2001
A female infant of nonconsanguineous Indian parents presented at 4 months with a hypoglycemic convulsion. Further episodes of… (More)
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Highly Cited
2000
Highly Cited
2000
Muscle contraction causes an increase in activity of 5'-AMP-activated protein kinase (AMPK). This study was designed to determine… (More)
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Highly Cited
1998
Highly Cited
1998
Very-long-chain acyl-CoA dehydrogenase (VLCAD) is an enzyme catalyzing the dehydrogenation of long-chain fatty acids in the first… (More)
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Highly Cited
1998
Highly Cited
1998
In aged mice, the redox-regulated transcription factor nuclear factor-kappaB (NF-kappaB) becomes constitutively active in many… (More)
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Highly Cited
1992
Highly Cited
1992
Peroxisome proliferators are a diverse group of chemicals, including several hypolipidaemic drugs, that activate a nuclear… (More)
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