(Q34321296)

1 August 2017

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title

Metabolite proofreading, a neglected aspect of intermediary metabolism (English)

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1 August 2017

author

Emile Van Schaftingen

object named as

Emile Van Schaftingen

1

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author name string

Rim Rzem

2

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1 August 2017

Alexandre Marbaix

3

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1 August 2017

François Collard

4

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1 August 2017

Maria Veiga-da-Cunha

5

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1 August 2017

Carole L Linster

6

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1 August 2017

publication date

8 January 2013

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Journal of Inherited Metabolic Disease

1 August 2017

published in

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1 August 2017

volume

36

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1 August 2017

issue

3

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1 August 2017

page(s)

427-434

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Equilibrium of 5,6-hydration of NADH and mechanism of ATP-dependent dehydration

1 August 2017

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Cloning, sequencing and expression of rat liver 3-phosphoglycerate dehydrogenase

21 January 2018

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Identification of a dehydrogenase acting on D-2-hydroxyglutarate

21 January 2018

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A novel GDP-D-glucose phosphorylase involved in quality control of the nucleoside diphosphate sugar pool in Caenorhabditis elegans and mammals

21 January 2018

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L-2-hydroxyglutaric acidemia: A novel inherited neurometabolic disease

21 January 2018

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Cancer-associated IDH1 mutations produce 2-hydroxyglutarate

21 January 2018

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Molecular identification of carnosine synthase as ATP-grasp domain-containing protein 1 (ATPGD1)

21 January 2018

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L-2-Hydroxyglutaric aciduria: an inborn error of metabolism?

21 January 2018

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Extending biochemical databases by metabolomic surveys

21 January 2018

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Biotransformation of L-lysine to L-pipecolic acid catalyzed by L-lysine 6-aminotransferase and pyrroline-5-carboxylate reductase

21 January 2018

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'Unknown' proteins and 'orphan' enzymes: the missing half of the engineering parts list--and how to find it

21 January 2018

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Identification of Escherichia coli YgaF as an L-2-hydroxyglutarate oxidase

21 January 2018

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Identification of the gene encoding hydroxyacid-oxoacid transhydrogenase, an enzyme that metabolizes 4-hydroxybutyrate

21 January 2018

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The aetiology of neurological complications of organic acidaemias--a role for the blood-brain barrier

21 January 2018

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IDH2 mutations in patients with D-2-hydroxyglutaric aciduria

21 January 2018

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Progress in understanding 2-hydroxyglutaric acidurias

21 January 2018

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Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading

21 January 2018

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Metabolite damage and its repair or pre-emption

21 January 2018

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Extremely conserved ATP- or ADP-dependent enzymatic system for nicotinamide nucleotide repair

21 January 2018

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IDH1 and IDH2 have critical roles in 2-hydroxyglutarate production in D-2-hydroxyglutarate dehydrogenase depleted cells

21 January 2018

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Mutations in antiquitin in individuals with pyridoxine-dependent seizures

21 January 2018

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Metabolism of ethylmalonate to mesaconate in the rat. Evidence for trans-dehydrogenation of methylsuccinate

21 January 2018

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Glyceraldehyde-3-phosphate dehydrogenase catalyzed hydration of the 5-6 double bond of reduced beta-nicotinamide adenine dinucleotide (betaNADH). Formation of beta-6-hydroxy-1,4,5,6-tetrahydronicotinamide adenine dinucleotide

21 January 2018

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A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria

21 January 2018

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The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase

21 January 2018

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L-2-hydroxyglutaric aciduria, a defect of metabolite repair

21 January 2018

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Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia

21 January 2018

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Identification of unknown protein function using metabolite cocktail screening

21 January 2018

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Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias

21 January 2018

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Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause D-2-hydroxyglutaric aciduria

21 January 2018

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Novel insights into L-2-hydroxyglutaric aciduria: mass isotopomer studies reveal 2-oxoglutaric acid as the metabolic precursor of L-2-hydroxyglutaric acid

21 January 2018

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Metabolism of lysine in alpha-aminoadipic semialdehyde dehydrogenase-deficient fibroblasts: evidence for an alternative pathway of pipecolic acid formation

21 January 2018

1 reference

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L-2-Hydroxyglutaric aciduria: identification of a mutant gene C14orf160, localized on chromosome 14q22.1

21 January 2018

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L: -2-Hydroxyglutaric aciduria, a disorder of metabolite repair

21 January 2018

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Identification of additional IDH mutations associated with oncometabolite R(-)-2-hydroxyglutarate production

21 January 2018

1 reference

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The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting alpha-ketoglutarate to 2-hydroxyglutarate

21 January 2018

1 reference

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The oxidation of l(-)alpha-hydroxyglutaric acid in animal tissues

21 January 2018

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Oncometabolite 2-hydroxyglutarate is a competitive inhibitor of α-ketoglutarate-dependent dioxygenases

21 January 2018

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Inhibition of NADP-dependent dehydrogenases by modified products of NADPH.

21 January 2018

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A novel alpha-ketoglutarate reductase activity of the serA-encoded 3-phosphoglycerate dehydrogenase of Escherichia coli K-12 and its possible implications for human 2-hydroxyglutaric aciduria

21 January 2018

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Isolation and identification of Nα-(β-alanyl)lysine and Nα-(γ-aminobutyryl) lysine from bovine brain

21 January 2018

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12 December 2020

Identification of α-(β-alanyl)-lysine in rabbit muscle

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12 December 2020

Studies on the mechanism of fatty acid synthesis. XII. Acetyl coenzyme A carboxylase

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12 December 2020

Enzymatic conversion of a reduced diphosphopyridine nucleotide derivative to reduced diphosphopyridine nucleotide

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12 December 2020

Metabolism of propionic acid in animal tissues. VIII. Crystalline propionyl carboxylase

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12 December 2020

Effect of NADH-X on cytosolic glycerol-3-phosphate dehydrogenase

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12 December 2020

The action of glyceraldehyde-3-phosphate dehydrogenase on reduced diphosphopyridine nucleotide

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12 December 2020

10.1007/S10545-012-9571-1

Identifiers

DOI

1 reference

Dimensions Publication ID

1 August 2017

1010091340

0 references

1 reference