Variable effects of maturity-onset-diabetes-of-youth (MODY)-associated glucokinase mutations on substrate interactions and stability of the enzyme.
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Effects of novel maturity-onset diabetes of the young (MODY)-associated mutations on glucokinase activity and protein stabilityHomotropic allosteric regulation in monomeric mammalian glucokinaseA panel of diverse assays to interrogate the interaction between glucokinase and glucokinase regulatory protein, two vital proteins in human diseaseNaturally occurring glucokinase mutations are associated with defects in posttranslational S-nitrosylation.A phospho-BAD BH3 helix activates glucokinase by a mechanism distinct from that of allosteric activatorsAnalysis of the co-operative interaction between the allosterically regulated proteins GK and GKRP using tryptophan fluorescenceInheritance of rare functional GCKR variants and their contribution to triglyceride levels in families.Identification and functional characterisation of novel glucokinase mutations causing maturity-onset diabetes of the young in Slovakia.Glucokinase (GCK) mutations in hyper- and hypoglycemia: maturity-onset diabetes of the young, permanent neonatal diabetes, and hyperinsulinemia of infancy.Thermal stability of glucokinase (GK) as influenced by the substrate glucose, an allosteric glucokinase activator drug (GKA) and the osmolytes glycerol and ureaGlucokinase (GCK) mutations and their characterization in MODY2 children of southern Italy.Phenotypic severity of homozygous GCK mutations causing neonatal or childhood-onset diabetes is primarily mediated through effects on protein stability.Functional analysis of human glucokinase gene mutations causing MODY2: exploring the regulatory mechanisms of glucokinase activity.Discovery of a novel site regulating glucokinase activity following characterization of a new mutation causing hyperinsulinemic hypoglycemia in humansGeneration of N-ethyl-N-nitrosourea (ENU) diabetes models in mice demonstrates genotype-specific action of glucokinase activators.Hyperinsulinism in infancy: from basic science to clinical disease.Correlation of rare coding variants in the gene encoding human glucokinase regulatory protein with phenotypic, cellular, and kinetic outcomes.GCK-MODY diabetes associated with protein misfolding, cellular self-association and degradation.Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations.Dominant form of congenital hyperinsulinism maps to HK1 region on 10q.Effect of mutations on the sensitivity of human beta-cell glucokinase to liver regulatory protein.Amino acid conservation in animal glucokinases. Identification of residues implicated in the interaction with the regulatory protein.Susceptibility of glucokinase-MODY mutants to inactivation by oxidative stress in pancreatic β-cells.Cell-biological assessment of human glucokinase mutants causing maturity-onset diabetes of the young type 2 (MODY-2) or glucokinase-linked hyperinsulinaemia (GK-HI).Structure-function analysis of the alpha5 and the alpha13 helices of human glucokinase: description of two novel activating mutations.The P446L variant in GCKR associated with fasting plasma glucose and triglyceride levels exerts its effect through increased glucokinase activity in liver.Catalytic activation of human glucokinase by substrate binding: residue contacts involved in the binding of D-glucose to the super-open form and conformational transitions.Structural instability of mutant beta-cell glucokinase: implications for the molecular pathogenesis of maturity-onset diabetes of the young (type-2).Human pancreatic glucokinase (GlkB) complements the glucose signalling defect of Saccharomyces cerevisiae hxk2 mutants.Insights into the structure and regulation of glucokinase from a novel mutation (V62M), which causes maturity-onset diabetes of the young.Insight into the biochemical characteristics of a novel glucokinase gene mutation.Tryptophan Fluorescence Yields and Lifetimes as a Probe of Conformational Changes in Human Glucokinase.
P2860
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P2860
Variable effects of maturity-onset-diabetes-of-youth (MODY)-associated glucokinase mutations on substrate interactions and stability of the enzyme.
description
1995 nî lūn-bûn
@nan
1995年の論文
@ja
1995年学术文章
@wuu
1995年学术文章
@zh-cn
1995年学术文章
@zh-hans
1995年学术文章
@zh-my
1995年学术文章
@zh-sg
1995年學術文章
@yue
1995年學術文章
@zh
1995年學術文章
@zh-hant
name
Variable effects of maturity-o ...... s and stability of the enzyme.
@en
type
label
Variable effects of maturity-o ...... s and stability of the enzyme.
@en
prefLabel
Variable effects of maturity-o ...... s and stability of the enzyme.
@en
P2093
P2860
P356
P1433
P1476
Variable effects of maturity-o ...... s and stability of the enzyme.
@en
P2093
F M Matschinsky
K Niswender
M A Magnuson
M A Permutt
Y Tanizawa
P2860
P304
P356
10.1042/BJ3090167
P407
P478
309 ( Pt 1)
P577
1995-07-01T00:00:00Z