Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene
about
Nerve tissue-specific (GLUD2) and housekeeping (GLUD1) human glutamate dehydrogenases are regulated by distinct allosteric mechanisms: implications for biologic functionAcute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndromeExpression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutationsDominant inheritance of sialuria, an inborn error of feedback inhibitionMutations in the human UDP-N-acetylglucosamine 2-epimerase gene define the disease sialuria and the allosteric site of the enzymeGain-of-function variant in GLUD2 glutamate dehydrogenase modifies Parkinson's disease onsetHyperinsulinemic Hypoglycemia - The Molecular MechanismsHyperinsulinemic Hypoglycemia in Infancy: Current Concepts in Diagnosis and ManagementThe Diagnosis and Management of Hyperinsulinaemic HypoglycaemiaMechanisms of amino acid-stimulated insulin secretion in congenital hyperinsulinismThe discovery of human of GLUD2 glutamate dehydrogenase and its implications for cell function in health and diseaseNovel Inhibitors Complexed with Glutamate Dehydrogenase: ALLOSTERIC REGULATION BY CONTROL OF PROTEIN DYNAMICSA novel mechanism of V-type zinc inhibition of glutamate dehydrogenase results from disruption of subunit interactions necessary for efficient catalysisGreen Tea Polyphenols Control Dysregulated Glutamate Dehydrogenase in Transgenic Mice by Hijacking the ADP Activation SiteMultiple Forms of Glutamate Dehydrogenase in Animals: Structural Determinants and Physiological ImplicationsMitochondrial regulation of β-cell function: maintaining the momentum for insulin releaseTransamination is required for {alpha}-ketoisocaproate but not leucine to stimulate insulin secretionHyperinsulinism and Beckwith-Wiedemann syndromeExercise induced hypoglycaemic hyperinsulinismDysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutationsMacrosomia and hyperinsulinaemic hypoglycaemia in patients with heterozygous mutations in the HNF4A geneInteraction of glutaric aciduria type 1-related glutaryl-CoA dehydrogenase with mitochondrial matrix proteinsSpecificity in beta cell expression of L-3-hydroxyacyl-CoA dehydrogenase, short chain, and potential role in down-regulating insulin releaseGlutamate dehydrogenase 1 and SIRT4 regulate glial developmentBranched-chain amino acid metabolon: interaction of glutamate dehydrogenase with the mitochondrial branched-chain aminotransferase (BCATm)Identification, cloning and expression of the mouse N-acetylglutamate synthase geneGlutamate: a truly functional amino acid.Mutations in UCP2 in congenital hyperinsulinism reveal a role for regulation of insulin secretionThe Rudolf Schoenheimer Centenary Lecture. Isotopes in nutrition research.Insulin secretion and insulin-producing tumorsDiazoxide-responsive hyperinsulinemic hypoglycemia caused by HNF4A gene mutations.Genetics of neonatal hyperinsulinism.Hyperinsulinism of infancy: towards an understanding of unregulated insulin release. European Network for Research into Hyperinsulinism in Infancy.Practical management of hyperinsulinism in infancyIons, genes and insulin release: from basic science to clinical disease. Based on the 1998 R. D. Lawrence Lecture.ATP-sensitive potassium channelopathies: focus on insulin secretionDynamic changes of the phosphoproteome in postmortem mouse brains.Congenital hyperinsulinism: current status and future perspectives.Interaction of ingested leucine with glycine on insulin and glucose concentrationsMolecular mechanisms of protein induced hyperinsulinaemic hypoglycaemia.
P2860
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P2860
Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene
description
1998 nî lūn-bûn
@nan
1998 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
1998 թվականի մայիսին հրատարակված գիտական հոդված
@hy
1998年の論文
@ja
1998年論文
@yue
1998年論文
@zh-hant
1998年論文
@zh-hk
1998年論文
@zh-mo
1998年論文
@zh-tw
1998年论文
@wuu
name
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@ast
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@en
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@nl
type
label
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@ast
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@en
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@nl
prefLabel
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@ast
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@en
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@nl
P2093
P3181
P1476
Hyperinsulinism and hyperammon ...... e glutamate dehydrogenase gene
@en
P2093
A B Burlina
C A Stanley
C R Greenberg
E Zammarchi
N J Hopwood
P304
P3181
P356
10.1056/NEJM199805073381904
P407
P577
1998-05-07T00:00:00Z