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

Related topics

Related topics

23 relations
2-Methylbutyryl-CoA Dehydrogenase DeficiencyACADS geneACADS wt AlleleAutosomal recessive inheritance
(More)

Broader (2)

Lipid Metabolism, Inborn Errorsacyl-CoA dehydrogenase

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2012
Highly Cited
2012
Clinical features, management, and prognosis of spontaneous coronary artery dissection.
BACKGROUND Spontaneous coronary artery dissection (SCAD) is an acute coronary event of uncertain origin. Clinical features and… (More)
  • table 1
  • table 2
  • table 3
  • table 1
  • figure 1
Is this relevant?
Highly Cited
2009
Highly Cited
2009
Sparsistency and Rates of Convergence in Large Covariance Matrix Estimation.
This paper studies the sparsistency and rates of convergence for estimating sparse covariance and precision matrices based on… (More)
Is this relevant?
Highly Cited
2008
Highly Cited
2008
Genetics Meets Metabolomics: A Genome-Wide Association Study of Metabolite Profiles in Human Serum
The rapidly evolving field of metabolomics aims at a comprehensive measurement of ideally all endogenous metabolites in a cell or… (More)
  • figure 1
  • figure 2
  • table 1
  • figure 3
  • table 2
Is this relevant?
Highly Cited
2007
Highly Cited
2007
Excess lipid availability increases mitochondrial fatty acid oxidative capacity in muscle: evidence against a role for reduced fatty acid oxidation in lipid-induced insulin resistance in rodents.
A reduced capacity for mitochondrial fatty acid oxidation in skeletal muscle has been proposed as a major factor leading to the… (More)
  • table 1
  • figure 1
  • figure 2
  • table 2
  • figure 3
Is this relevant?
Highly Cited
2005
Highly Cited
2005
Pioglitazone induces mitochondrial biogenesis in human subcutaneous adipose tissue in vivo.
Thiazolidenediones such as pioglitazone improve insulin sensitivity in diabetic patients by several mechanisms, including… (More)
  • figure 1
  • figure 2
  • figure 3
  • figure 4
Is this relevant?
Highly Cited
2001
Highly Cited
2001
Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion.
A female infant of nonconsanguineous Indian parents presented at 4 months with a hypoglycemic convulsion. Further episodes of… (More)
  • table 1
  • table 2
  • figure 1
  • table 3
  • table 4
Is this relevant?
Highly Cited
2000
Highly Cited
2000
Activation of AMP-activated protein kinase increases mitochondrial enzymes in skeletal muscle.
Muscle contraction causes an increase in activity of 5'-AMP-activated protein kinase (AMPK). This study was designed to determine… (More)
  • table 1
  • figure 1
  • table 2
  • figure 3
  • figure 2
Is this relevant?
Highly Cited
1998
Highly Cited
1998
Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients.
Very-long-chain acyl-CoA dehydrogenase (VLCAD) is an enzyme catalyzing the dehydrogenation of long-chain fatty acids in the first… (More)
Is this relevant?
Highly Cited
1998
Highly Cited
1998
Peroxisome proliferator-activated receptor alpha activation modulates cellular redox status, represses nuclear factor-kappaB signaling, and reduces inflammatory cytokine production in aging.
In aged mice, the redox-regulated transcription factor nuclear factor-kappaB (NF-kappaB) becomes constitutively active in many… (More)
  • figure 1
  • figure 2
  • figure 3
  • figure 5
  • figure 4
Is this relevant?
Highly Cited
1992
Highly Cited
1992
The mouse peroxisome proliferator activated receptor recognizes a response element in the 5' flanking sequence of the rat acyl CoA oxidase gene.
Peroxisome proliferators are a diverse group of chemicals, including several hypolipidaemic drugs, that activate a nuclear… (More)
Is this relevant?