Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase (Q34181596)

31 July 2017

title

Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase (English)

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Hydroxyacyl-Coenzyme A dehydrogenase

31 July 2017

main subject

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Glutamate dehydrogenase 1

GOA release 2020-03-11

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GOA release 2020-03-11

Thomas J Smith

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Noam A. Cohen

object named as

Noam A Cohen

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Charles A Stanley

object named as

Charles A Stanley

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author name string

Changhong Li

1

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31 July 2017

Pan Chen

2

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31 July 2017

Andrew Palladino

3

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31 July 2017

Srinivas Narayan

4

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31 July 2017

Laurie K Russell

5

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31 July 2017

Samir Sayed

6

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31 July 2017

Guoxiang Xiong

7

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31 July 2017

Jie Chen

8

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31 July 2017

David Stokes

9

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31 July 2017

Yasmeen M Butt

10

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31 July 2017

Patricia M Jones

11

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31 July 2017

Heather W Collins

12

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31 July 2017

Akiva S Cohen

14

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31 July 2017

Itzhak Nissim

15

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31 July 2017

Arnold W Strauss

17

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31 July 2017

Franz M Matschinsky

18

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31 July 2017

Michael J Bennett

19

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31 July 2017

language of work or name

English

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publication date

29 July 2010

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Journal of Biological Chemistry

31 July 2017

published in

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31 July 2017

volume

285

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31 July 2017

issue

41

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31 July 2017

page(s)

31806-31818

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On the reversibility of glutamate dehydrogenase and the source of hyperammonemia in the hyperinsulinism/hyperammonemia syndrome

31 July 2017

cites work

1 reference

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5 July 2018

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Elimination of KATP channels in mouse islets results in elevated [U-13C]glucose metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma-aminobutyric acid shunt

5 July 2018

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Specificity in beta cell expression of L-3-hydroxyacyl-CoA dehydrogenase, short chain, and potential role in down-regulating insulin release

5 July 2018

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5 July 2018

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SIRT4 inhibits glutamate dehydrogenase and opposes the effects of calorie restriction in pancreatic beta cells

5 July 2018

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Reye-like syndrome resulting from novel missense mutations in mitochondrial medium- and short-chain l-3-hydroxy-acyl-CoA dehydrogenase

5 July 2018

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5 July 2018

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Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism

5 July 2018

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5 July 2018

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Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation

5 July 2018

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5 July 2018

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Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion

5 July 2018

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Tissue-specific deletion of Foxa2 in pancreatic beta cells results in hyperinsulinemic hypoglycemia

5 July 2018

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5 July 2018

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Improved stable isotope dilution-gas chromatography-mass spectrometry method for serum or plasma free 3-hydroxy-fatty acids and its utility for the study of disorders of mitochondrial fatty acid beta-oxidation

5 July 2018

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5 July 2018

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Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene

5 July 2018

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5 July 2018

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Short-chainL-3-hydroxyacyl-CoA dehydrogenase deficiency in muscle: A new cause for recurrent myoglobinuria and encephalopathy

5 July 2018

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Hyperinsulinism of infancy associated with a novel splice site mutation in the SCHAD gene.

5 July 2018

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3-Hydroxyacyl-coenzyme A dehydrogenase deficiency and hyperinsulinemic hypoglycemia: characterization of a novel mutation and severe dietary protein sensitivity.

21 January 2018

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Regulation of leucine-stimulated insulin secretion and glutamine metabolism in isolated rat islets

21 January 2018

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Green tea polyphenols modulate insulin secretion by inhibiting glutamate dehydrogenase

21 January 2018

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Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency

21 January 2018

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Mitochondrial short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: a new defect of fatty acid oxidation

21 January 2018

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Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K currents.

21 January 2018

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Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. HI/HA Contributing Investigators

21 January 2018

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Ketotic Episodes in Glutaryl-CoA Dehydrogenase Deficiency (Glutaric Aciduria)

21 January 2018

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12 December 2020

Nutrient secretagogues induce bimodal early changes in cytoplasmic calcium of insulin-releasing ob/ob mouse beta-cells

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12 December 2020

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

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12 December 2020

Fatal hepatic short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: clinical, biochemical, and pathological studies on three subjects with this recently identified disorder of mitochondrial beta-oxidation

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Effects of a GTP-insensitive Mutation of Glutamate Dehydrogenase on Insulin Secretion in Transgenic Mice

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12 December 2020

Identifiers

DOI

10.1074/JBC.M110.123638

1 reference

31 July 2017

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31 July 2017

1 reference