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Article

Structural instability of mutant beta-cell glucokinase: implications for the molecular pathogenesis of maturity-onset diabetes of the young (type-2).

The Biochemical journal - 15 Feb 1997

Kesavan P, Wang L, Davis E, Cuesta A, Sweet I, Niswender K, Magnuson M A, Matschinsky F M

Abstract excerpt

The catalytic function and thermal stability of wild-type and mutant recombinant human pancreatic beta-cell glucokinase was investigated. The mutants E70K and E300K, which are thought to be the cause of impaired insulin production by the pancreatic beta-cell and decreased glucose uptake by the liver of patients with maturity-onset diabetes of the young, were found to be functionally indistinguishable from the...

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Identifiers

PMID: 9078243
DOI: 10.1042/bj3220057
PMCID: PMC1218158

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Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Structural instability of mutant beta-cell glucokinase: implications for the molecular pathogenesis of maturity-onset diabetes of the young (type-2).PMID 9078243Read ↗

From clinicogenetic studies of maturity-onset diabetes of the young to unraveling complex mechanisms of glucokinase regulation.Semantic proximity Read ↗

Characterization of glucokinase mutations associated with maturity-onset diabetes of the young type 2 (MODY-2): different glucokinase defects lead to a common phenotype.Semantic proximity Read ↗

GCK-MODY diabetes as a protein misfolding disease: the mutation R275C promotes protein misfolding, self-association and cellular degradation.Semantic proximity Read ↗

Phenotypic severity of homozygous GCK mutations causing neonatal or childhood-onset diabetes is primarily mediated through effects on protein stability.Semantic proximity Read ↗

GCK-MODY diabetes associated with protein misfolding, cellular self-association and degradation.Semantic proximity Read ↗

Susceptibility of glucokinase-MODY mutants to inactivation by oxidative stress in pancreatic β-cells.Semantic proximity Read ↗

Effects of novel maturity-onset diabetes of the young (MODY)-associated mutations on glucokinase activity and protein stability.Semantic proximity Read ↗

Biochemical Basis of Glucokinase Activation and the Regulation by Glucokinase Regulatory Protein in Naturally Occurring MutationsSemantic proximity Read ↗

Thermal stability of glucokinase (GK) as influenced by the substrate glucose, an allosteric glucokinase activator drug (GKA) and the osmolytes glycerol and urea.Semantic proximity Read ↗

[The functional analysis of glucokinase gene E339K mutation].Semantic proximity Read ↗

Mutants of glucokinase cause hypoglycaemia- and hyperglycaemia syndromes and their analysis illuminates fundamental quantitative concepts of glucose homeostasis.Semantic proximity Read ↗

Glucokinase: structural analysis of a protein involved in susceptibility to diabetes.Semantic proximity Read ↗

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