Congenital hyperinsulinism due to mutations in HNF4A and HADH. (Q37798750)

23 August 2017

Congenital hyperinsulinism due to mutations in HNF4A and HADH. (English)

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23 August 2017

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2

Amanda Heslegrave

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23 August 2017

Ritika R Kapoor

1

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PubMed ID

20931292

23 August 2017

Khalid Hussain

series ordinal

3

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23 August 2017

1 September 2010

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Reviews in Endocrine and Metabolic Disorders

23 August 2017

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23 August 2017

11

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23 August 2017

3

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23 August 2017

185-191

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https://scigraph.springernature.com/pub.10.1007/s11154-010-9148-y

23 August 2017

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cites work

Mammalian mitochondrial beta-oxidation

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Foxa2 regulates multiple pathways of insulin secretion.

21 January 2018

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

21 January 2018

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https://api.crossref.org/works/10.1007%2FS11154-010-9148-Y

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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12 December 2020

Diazoxide-responsive hyperinsulinemic hypoglycemia caused by HNF4A gene mutations

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12 December 2020

Hunting for a hypoglycemia gene: severe neonatal hypoglycemia in a consanguineous family

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12 December 2020

Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by KATP channel mutations

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Persistent hyperinsulinemic hypoglycemia and maturity-onset diabetes of the young due to heterozygous HNF4A mutations

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12 December 2020

Pancreatic islet adaptation to fasting is dependent on peroxisome proliferator-activated receptor alpha transcriptional up-regulation of fatty acid oxidation

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12 December 2020

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

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12 December 2020

A transcription factor regulatory circuit in differentiated pancreatic cells

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12 December 2020

A distant upstream promoter of the HNF-4alpha gene connects the transcription factors involved in maturity-onset diabetes of the young

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12 December 2020

L-3-hydroxyacyl coenzyme A dehydrogenase from pig heart muscle. I. Purification and properties

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12 December 2020

Macrosomia and neonatal hypoglycaemia in RW pedigree subjects with a mutation (Q268X) in the gene encoding hepatocyte nuclear factor 4alpha (HNF4A)

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12 December 2020

Epistasis of transcriptomes reveals synergism between transcriptional activators Hnf1alpha and Hnf4alpha

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12 December 2020

Hepatocyte nuclear factor 4alpha regulates the expression of pancreatic beta -cell genes implicated in glucose metabolism and nutrient-induced insulin secretion

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12 December 2020

Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence

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12 December 2020

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

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12 December 2020

Liver-enriched transcription factor HNF-4 is a novel member of the steroid hormone receptor superfamily

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12 December 2020

Assay of L-3-hydroxyacyl-coenzyme A dehydrogenase with substrates of different chain lengths

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12 December 2020

The MODY1 gene HNF-4alpha regulates selected genes involved in insulin secretion

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12 December 2020

Hepatocyte nuclear factor-4alpha is essential for glucose-stimulated insulin secretion by pancreatic beta-cells

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12 December 2020

Structural organization of the human short-chain L-3-hydroxyacyl-CoA dehydrogenase gene

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12 December 2020

Control of pancreas and liver gene expression by HNF transcription factors

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12 December 2020

Mutations in the hepatocyte nuclear factor-4alpha gene in maturity-onset diabetes of the young (MODY1)

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12 December 2020

Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with hyperinsulinism: a novel glucose-fatty acid cycle?

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12 December 2020

Familial hypoglycemia precipitated by amino acids

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12 December 2020

Fatty acid oxidation disorders

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12 December 2020

Identification of a diffuse form of hyperinsulinemic hypoglycemia by 18-fluoro-L-3,4 dihydroxyphenylalanine positron emission tomography/CT in a patient carrying a novel mutation of the HADH gene.

1 reference

12 December 2020

Malonyl-CoA signaling, lipid partitioning, and glucolipotoxicity: role in beta-cell adaptation and failure in the etiology of diabetes

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12 December 2020

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

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12 December 2020

Regulation of insulin release by factors that also modify glutamate dehydrogenase

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12 December 2020

Macrosomia and hyperinsulinaemic hypoglycaemia in patients with heterozygous mutations in the HNF4A gene

1 reference

12 December 2020

Hyperinsulinaemic hypoglycaemia.

1 reference

12 December 2020

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

1 reference

12 December 2020

Hydroxyacyl CoA dehydrogenase, an enzyme important in fat metabolism in different cell types in the islets of Langerhans

1 reference

12 December 2020

Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency

1 reference

12 December 2020

10.1007/S11154-010-9148-Y

Identifiers

DOI

1 reference

23 August 2017

1 reference

PubMed ID

20931292