Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects
about
Mechanisms of pharmacological rescue of trafficking-defective hERG mutant channels in human long QT syndromeRescue of aberrant gating by a genetically encoded PAS (Per-Arnt-Sim) domain in several long QT syndrome mutant human ether-á-go-go-related gene potassium channelsVoltage-gated Nav channel targeting in the heart requires an ankyrin-G dependent cellular pathwayMexiletine differentially restores the trafficking defects caused by two brugada syndrome mutationsTranslational toxicology and rescue strategies of the hERG channel dysfunction: biochemical and molecular mechanistic aspectsThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyMolecular pathogenesis of long QT syndrome type 2Variants of Transient Receptor Potential Melastatin Member 4 in Childhood Atrioventricular BlockDifferent roles for the cyclic nucleotide binding domain and amino terminus in assembly and expression of hyperpolarization-activated, cyclic nucleotide-gated channelsTrafficking defect and proteasomal degradation contribute to the phenotype of a novel KCNH2 long QT syndrome mutationRestoration of proper trafficking to the cell surface for membrane proteins harboring cysteine mutationsPharmacologic Approach to Defective Protein Trafficking in the E637K-hERG Mutant with PD-118057 and ThapsigarginExpression and role of the ether-à-go-go-related (MERG1A) potassium-channel protein during preimplantation mouse developmentCharacterization of Dent's disease mutations of CLC-5 reveals a correlation between functional and cell biological consequences and protein structure.Spinocerebellar ataxia type 19/22 mutations alter heterocomplex Kv4.3 channel function and gating in a dominant mannerInteraction with GM130 during HERG ion channel trafficking. Disruption by type 2 congenital long QT syndrome mutations. Human Ether-à-go-go-Related Gene.Novel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome.Electrophysiological Characteristics of the LQT2 Syndrome Mutation KCNH2-G572S and Regulation by Accessory Protein KCNE2.Improved functional expression of recombinant human ether-a-go-go (hERG) K+ channels by cultivation at reduced temperatureIatrogenic QT Abnormalities and Fatal Arrhythmias: Mechanisms and Clinical SignificancePharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum.A Drosophila behavioral mutant, down and out (dao), is defective in an essential regulator of Erg potassium channels.Using pharmacological chaperones to restore proteostasisHERG1 channelopathiesProperties of WT and mutant hERG K(+) channels expressed in neonatal mouse cardiomyocytes.Chaperoning G protein-coupled receptors: from cell biology to therapeuticsTrafficking-deficient G572R-hERG and E637K-hERG activate stress and clearance pathways in endoplasmic reticulum.Intracellular regions of the Eag potassium channel play a critical role in generation of voltage-dependent currents.Defective trafficking and function of KATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy.Selective knockout of mouse ERG1 B potassium channel eliminates I(Kr) in adult ventricular myocytes and elicits episodes of abrupt sinus bradycardia.Chemical chaperones: a pharmacological strategy for disorders of protein folding and trafficking.Protein trafficking abnormalities: a new mechanism in drug-induced long QT syndrome.Protein kinase A activity at the endoplasmic reticulum surface is responsible for augmentation of human ether-a-go-go-related gene product (HERG).Trafficking-deficient hERG K⁺ channels linked to long QT syndrome are regulated by a microtubule-dependent quality control compartment in the ER.Mouse ERG K(+) channel clones reveal differences in protein trafficking and function.Improved functional expression of human cardiac kv1.5 channels and trafficking-defective mutants by low temperature treatmentRegulation of ABCC6 trafficking and stability by a conserved C-terminal PDZ-like sequencePartially dominant mutant channel defect corresponding with intermediate LQT2 phenotype.The use of Bcl-2 over-expression to stabilize hybridomas specific to the HERG potassium channelSimple image-based no-wash method for quantitative detection of surface expressed CFTR.
P2860
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P2860
Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects
description
1999 nî lūn-bûn
@nan
1999 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
name
Correction of defective protei ...... ogical and temperature effects
@ast
Correction of defective protei ...... ogical and temperature effects
@en
Correction of defective protei ...... ogical and temperature effects
@nl
type
label
Correction of defective protei ...... ogical and temperature effects
@ast
Correction of defective protei ...... ogical and temperature effects
@en
Correction of defective protei ...... ogical and temperature effects
@nl
prefLabel
Correction of defective protei ...... ogical and temperature effects
@ast
Correction of defective protei ...... ogical and temperature effects
@en
Correction of defective protei ...... ogical and temperature effects
@nl
P2093
P2860
P3181
P356
P1476
Correction of defective protei ...... ogical and temperature effects
@en
P2093
P2860
P304
P3181
P356
10.1074/JBC.274.44.31123
P407
P577
1999-10-29T00:00:00Z