Very long chain acyl-CoA dehydrogenase deficiency

Known as: Acyl-CoA Dehydrogenase, Very Long-Chain, Deficiency of, Very Long-Chain Acyl Coenzyme A Dehydrogenase Deficiency, Sideroblastic anemia with marrow cell vacuolization and exocrine pancreatic dysfunction 
An autosomal recessive inherited disorder characterized by a deficiency of the enzyme very long-chain acyl-coenzyme A dehydrogenase that metabolizes… (More)
National Institutes of Health

Papers overview

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2014
2014
Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) is a fatty acid oxidation disorder with widely varying presentations… (More)
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2010
2010
Mitochondrial fatty acid oxidation provides an important energy source for cellular metabolism, and decreased mitochondrial fatty… (More)
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2009
2009
Cardiac hypertrophy is a common finding in human patients with inborn errors of long-chain fatty acid oxidation. Mice with either… (More)
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2009
2009
BACKGROUND Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD, respectively) deficiency… (More)
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2006
2006
OBJECTIVE Neonatal screening programs for very long-chain acyl-coenzyme A dehydrogenase deficiency have been implemented recently… (More)
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Highly Cited
2001
Highly Cited
2001
Although many patients have been found to have very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, none have been… (More)
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2001
2001
A patient with very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is reported. He had a severe neonatal presentation and… (More)
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Highly Cited
1999
Highly Cited
1999
Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the initial rate-limiting step in mitochondrial fatty acid beta… (More)
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Highly Cited
1995
Highly Cited
1995
beta-Oxidation of long-chain fatty acids provides the major source of energy in the heart. Defects in enzymes of the beta… (More)
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1993
1993
ABSTRACT: Long-chain acyl-CoA dehydrogenase (LCAD) deficiency is a disorder of fatty acid β-oxidation. Its diagnosis has been… (More)
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