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Isovaleryl-CoA dehydrogenase

Known as: Isovaleryl CoA Dehydrogenase, Isovaleryl-Coenzyme A Dehydrogenase, CoA Dehydrogenase, Isovaleryl 
A mitochondrial flavoprotein, this enzyme catalyzes the oxidation of 3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA using FAD as a cofactor. Defects… (More)
National Institutes of Health

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Topic mentions per year

1980-2015
01219802015

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IVD geneIn BloodProcess of secretionadministration & dosage
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Isovaleryl-CoA dehydrogenase deficiency

Related mentions per year

Related mentions per year

1936-2018
19401960198020002020
Isovaleryl-CoA dehydrogenase
physiological aspects
chemical synthesis
Isovaleryl-CoA dehydrogenase deficiency
IVD gene
standards characteristics

Papers overview

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2005
2005
Establishment of a practical enzymatic assay method for determination of isovaleryl-CoA dehydrogenase activity using high-performance liquid chromatography.
BACKGROUND Isovaleric acidemia (IVA) is one of the various target disorders for tandem mass spectrometry (MS/MS) newborn… (More)
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2001
2001
Identification of Caenorhabditis elegans isovaleryl-CoA dehydrogenase and structural comparison with other acyl-CoA dehydrogenases.
Isovaleryl-CoA dehydrogenase (IVD) is a flavoenzyme, which catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA in… (More)
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2001
2001
Cloning of genomic and cDNA for mouse isovaleryl-CoA dehydrogenase (IVD) and evolutionary comparison to other known IVDs.
Isovaleryl-CoA dehydrogenase (IVD) is an intramitochondrial homotetrameric flavoenzyme that catalyzes the conversion of… (More)
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2000
2000
Cloning of a gene for an acyl-CoA dehydrogenase from Pisum sativum L. and purification and characterization of its product as an isovaleryl-CoA dehydrogenase.
Isovaleryl-CoA dehydrogenase (IVD, EC ) catalyzes the third step in the catabolism of leucine in mammals. Deficiency of this… (More)
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1998
1998
Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia.
Isovaleryl-CoA dehydrogenase (IVD) is a homotetrameric mitochondrial flavoenzyme which catalyzes the conversion of isovaleryl-CoA… (More)
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1997
1997
Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,.
Isovaleryl-CoA dehydrogenase (IVD) belongs to an important flavoprotein family of acyl-CoA dehydrogenases that catalyze the alpha… (More)
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1995
1995
High-level expression of an altered cDNA encoding human isovaleryl-CoA dehydrogenase in Escherichia coli.
Isovaleryl-CoA dehydrogenase (IVD) catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA in the leucine catabolism… (More)
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1995
1995
Identification of the active site catalytic residue in human isovaleryl-CoA dehydrogenase.
Isovaleryl-CoA dehydrogenase (IVD) is a homotetrameric flavoenzyme which catalyzes the conversion of isovaleryl-CoA to 3… (More)
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1993
1993
Structural organization of the human isovaleryl-CoA dehydrogenase gene.
Isovaleryl-CoA dehydrogenase (IVD) catalyzes the third step in leucine catabolism. The genetic deficiency of IVD causes… (More)
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1986
1986
Isovaleryl-CoA Dehydrogenase Activity in Isovaleric Acidemia Fibroblasts Using an Improved Tritium Release Assay
ABSTRACT. Isovaleric acidemia is a disorder of leucine metabolism caused by a deficiency of isovaleryl-CoA dehydrogenase. At… (More)
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