Mutant INS-gene induced diabetes of youth: proinsulin cysteine residues impose dominant-negative inhibition on wild-type proinsulin transport - Wikidata - awgldk - TriplyDB

Mutant INS-gene induced diabetes of youth: proinsulin cysteine residues impose dominant-negative inhibition on wild-type proinsulin transport

Mutant INS-gene induced diabetes of youth: proinsulin cysteine residues impose dominant-negative inhibition on wild-type proinsulin transport

http://www.wikidata.org/entity/Q33719171

about

INS-gene mutations: from genetics and beta cell biology to clinical disease Neonatal diabetes: an expanding list of genes allows for improved diagnosis and treatment Insulin analog with additional disulfide bond has increased stability and preserved activity Insight into the Structural and Biological Relevance of the T/R Transition of the N-Terminus of the B-Chain in Human Insulin Protective hinge in insulin opens to enable its receptor engagement Stimulation of autophagy improves endoplasmic reticulum stress-induced diabetes Structural and functional study of the GlnB22-insulin mutant responsible for maturity-onset diabetes of the young Aromatic anchor at an invariant hormone-receptor interface: function of insulin residue B24 with application to protein design.Transgenic zebrafish model of the C43G human insulin gene mutation Inefficient translocation of preproinsulin contributes to pancreatic β cell failure and late-onset diabetes.Hyperglucagonemia in an animal model of insulin- deficient diabetes: what therapy can improve it?Permanent neonatal diabetes caused by creation of an ectopic splice site within the INS gene.SORCS1 is necessary for normal insulin secretory granule biogenesis in metabolically stressed β cells.Proinsulin misfolding and endoplasmic reticulum stress during the development and progression of diabetes.Action of protein disulfide isomerase on proinsulin exit from endoplasmic reticulum of pancreatic β-cells.Proteasomal Degradation of Proinsulin Requires Derlin-2, HRD1 and p97.Impaired cleavage of preproinsulin signal peptide linked to autosomal-dominant diabetes PDI reductase acts on Akita mutant proinsulin to initiate retrotranslocation along the Hrd1/Sel1L-p97 axis.Islet autoantigens: structure, function, localization, and regulation.A Bystander Mechanism Explains the Specific Phenotype of a Broadly Expressed Misfolded Protein.Competitive Inhibition of the Endoplasmic Reticulum Signal Peptidase by Non-cleavable Mutant Preprotein Cargos Proinsulin intermolecular interactions during secretory trafficking in pancreatic β cells Disulfide Mispairing During Proinsulin Folding in the Endoplasmic Reticulum.Monogenic Diabetes: What It Teaches Us on the Common Forms of Type 1 and Type 2 Diabetes.Dominant protein interactions that influence the pathogenesis of conformational diseases.Diabetes mellitus due to the toxic misfolding of proinsulin variants.Permanent neonatal diabetes due to a novel insulin signal peptide mutation.Endoplasmic reticulum oxidoreductin-1α (Ero1α) improves folding and secretion of mutant proinsulin and limits mutant proinsulin-induced endoplasmic reticulum stress.Genetic complexity in a Drosophila model of diabetes-associated misfolded human proinsulin.Calcium efflux from the endoplasmic reticulum leads to β-cell death.Endoplasmic reticulum stress and pancreatic β-cell death.Autophagy in adipose tissue and the beta cell: implications for obesity and diabetes.Insulin gene mutations and diabetes Chaperone-Driven Degradation of a Misfolded Proinsulin Mutant in Parallel With Restoration of Wild-Type Insulin Secretion.Aberrant disulphide bonding contributes to the ER retention of alpha1-antitrypsin deficiency variants.Cysteines as Redox Molecular Switches and Targets of Disease.C-peptide evolution: generation from few structural restrictions of bioactivities not necessarily functional.Endoplasmic reticulum stress and eIF2α phosphorylation: The Achilles heel of pancreatic β cells.Evaluation of conformational changes in diabetes-associated mutation in insulin a chain: a molecular dynamics study.[Endoplasmic reticulum stress: from physiology to pathogenesis of type 2 diabetes].

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Mutant INS-gene induced diabetes of youth: proinsulin cysteine residues impose dominant-negative inhibition on wild-type proinsulin transport

http://www.wikidata.org/entity/Q33719171

description

2010 nî lūn-bûn

@nan

2010 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած

@hyw

2010 թվականի հոտեմբերին հրատարակված գիտական հոդված

@hy

2010年の論文

@ja

2010年論文

@yue

2010年論文

@zh-hant

2010年論文

@zh-hk

2010年論文

@zh-mo

2010年論文

@zh-tw

2010年论文

@wuu

name

Mutant INS-gene induced diabet ...... wild-type proinsulin transport

@ast

Mutant INS-gene induced diabet ...... wild-type proinsulin transport

@en

type

label

Mutant INS-gene induced diabet ...... wild-type proinsulin transport

@ast

Mutant INS-gene induced diabet ...... wild-type proinsulin transport

@en

prefLabel

Mutant INS-gene induced diabet ...... wild-type proinsulin transport

@ast

Mutant INS-gene induced diabet ...... wild-type proinsulin transport

@en

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JOURNAL.PONE.0013333

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Mutant INS-gene induced diabet ...... wild-type proinsulin transport

@en

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Leena Haataja

http://www.w3.org/2001/XMLSchema#string

Michael A Weiss

http://www.w3.org/2001/XMLSchema#string

Ming Liu

http://www.w3.org/2001/XMLSchema#string

Nelson F Phillips

http://www.w3.org/2001/XMLSchema#string

Peter Arvan

http://www.w3.org/2001/XMLSchema#string

Qing-Xin Hua

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P2860

Mammalian transcription factor ATF6 is synthesized as a transmembrane protein and activated by proteolysis in response to endoplasmic reticulum stress

A stress response pathway from the endoplasmic reticulum to the nucleus requires a novel bifunctional protein kinase/endoribonuclease (Ire1p) in mammalian cells

Induction of Grp78/BiP by translational block: activation of the Grp78 promoter by ATF4 through and upstream ATF/CRE site independent of the endoplasmic reticulum stress elements

IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response

Chiral mutagenesis of insulin's hidden receptor-binding surface: structure of an allo-isoleucine(A2) analogue

Enhancing the Activity of a Protein by Stereospecific Unfolding: CONFORMATIONAL LIFE CYCLE OF INSULIN AND ITS EVOLUTIONARY ORIGINS

Paradoxical structure and function in a mutant human insulin associated with diabetes mellitus

Mapping the functional surface of insulin by design: structure and function of a novel A-chain analogue

The ERO1 gene of yeast is required for oxidation of protein dithiols in the endoplasmic reticulum.

Control of mRNA translation preserves endoplasmic reticulum function in beta cells and maintains glucose homeostasis

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scholarly article

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10.1371/JOURNAL.PONE.0013333

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10

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5

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Fabrizio Barbetti

Nalinda P Wickramasinghe

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2010-10-11T00:00:00Z

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47430455

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20948967

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2952628

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