(Q28116825)

4 January 2017

title

Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans (English)

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4 January 2017

main subject

isobutyryl-coenzyme A

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ChEBI release 2020-05-28

Nasser Abd-El Razik

5

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Brage Storstein Andresen

object named as

Brage S. Andresen

2

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Jerry Vockley

object named as

Jerry Vockley

11

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author name string

Tien V. Nguyen

1

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4 January 2017

Thomas J. Corydon

3

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4 January 2017

Sandro Ghisla

4

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4 January 2017

Al-Walid A. Mohsen

6

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4 January 2017

Stephen D. Cederbaum

7

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4 January 2017

Diane S. Roe

8

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4 January 2017

Charles R. Roe

9

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4 January 2017

Nicolas J. Lench

10

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4 January 2017

language of work or name

English

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publication date

1 October 2016

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Molecular Genetics and Metabolism

4 January 2017

published in

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4 January 2017

volume

77

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4 January 2017

issue

1-2

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4 January 2017

page(s)

68–79

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Spectroscopic analysis of the interaction of rat liver short-chain, medium-chain, and long-chain acyl coenzyme A dehydrogenases with acyl coenzyme A substrates

4 January 2017

cites work

1 reference

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7 January 2021

Isolation and expression of a cDNA encoding the precursor for a novel member (ACADSB) of the acyl-CoA dehydrogenase gene family

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7 January 2021

Cloning of a cDNA for short/branched chain acyl-Coenzyme A dehydrogenase from rat and characterization of its tissue expression and substrate specificity

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7 January 2021

Inborn errors of mitochondrial fatty acid oxidation

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7 January 2021

Disorders of mitochondrial fatty acyl-CoA beta-oxidation

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7 January 2021

Identification of the catalytic residue of human short/branched chain acyl-CoA dehydrogenase by in vitro mutagenesis

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7 January 2021

2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism

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7 January 2021

Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and [...]

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7 January 2021

Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism

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7 January 2021

Isolation and characterisation of a cDNA encoding the precursor for a novel member of the acyl-CoA dehydrogenase gene family

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7 January 2021

Parameters affecting the yield of DNA from human blood

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7 January 2021

Functional role of the active site glutamate-368 in rat short chain acyl-CoA dehydrogenase

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7 January 2021

Identification of the Active Site Catalytic Residue in Human Isovaleryl-CoA Dehydrogenase

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7 January 2021

High-level expression of an altered cDNA encoding human isovaleryl-CoA dehydrogenase in Escherichia coli

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7 January 2021

Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia

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7 January 2021

Human and mouse mitochondrial orthologs of bacterial ClpX

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7 January 2021

Mammalian branched-chain acyl-CoA dehydrogenases: molecular cloning and characterization of recombinant enzymes

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7 January 2021

Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,

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7 January 2021

Structure of the medium-chain acyl-CoA dehydrogenase from pig liver mitochondria at 3-A resolution

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7 January 2021

Crystal structures of medium-chain acyl-CoA dehydrogenase from pig liver mitochondria with and without substrate

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7 January 2021

Three-dimensional structure of butyryl-CoA dehydrogenase from Megasphaera elsdenii

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7 January 2021

Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene

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7 January 2021

The mutational spectrum in very long-chain acyl-CoA dehydrogenase deficiency

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7 January 2021

Molecular evolution and substrate specificity of acyl-CoA dehydrogenases: chimaeric "medium/long' chain-specific enzyme from medium-chain acyl-CoA dehydrogenase

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7 January 2021

10.1016/S1096-7192(02)00152-X

Identifiers

DOI

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