HERG channel dysfunction in human long QT syndrome. Intracellular transport and functional defects.
about
Differential association between HERG and KCNE1 or KCNE214-3-3 amplifies and prolongs adrenergic stimulation of HERG K+ channel activityMechanisms 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 channelsInduced pluripotent stem cells as a disease modeling and drug screening platformThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyStructure of the carboxy-terminal region of a KCNH channelStructure of the C-terminal region of an ERG channel and functional implicationsMolecular pathogenesis of long QT syndrome type 2Expression and coassociation of ERG1, KCNQ1, and KCNE1 potassium channel proteins in horse heartCorrection of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effectsPharmacologic 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 developmentKv2.1 ablation alters glucose-induced islet electrical activity, enhancing insulin secretionAbsence of direct cyclic nucleotide modulation of mEAG1 and hERG1 channels revealed with fluorescence and electrophysiological methodsInteraction with GM130 during HERG ion channel trafficking. Disruption by type 2 congenital long QT syndrome mutations. Human Ether-à-go-go-Related Gene.Inherited long QT syndromes: a paradigm for understanding arrhythmogenesis.Novel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome.Variable expression of long QT syndrome among gene carriers from families with five different HERG mutations.Functional characterization of a trafficking-defective HCN4 mutation, D553N, associated with cardiac arrhythmia.Inherited and acquired vulnerability to ventricular arrhythmias: cardiac Na+ and K+ channels.Mouse models of long QT syndrome.A new C-terminal hERG mutation A915fs+47X associated with symptomatic LQT2 and auditory-trigger syncopeAn Interdomain KCNH2 Mutation Produces an Intermediate Long QT SyndromeAssociation of the hERG mutation with long-QT syndrome type 2, syncope and epilepsy.Iatrogenic QT Abnormalities and Fatal Arrhythmias: Mechanisms and Clinical SignificanceHsp40 chaperones promote degradation of the HERG potassium channel.Pharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum.The EGFP/hERG fusion protein alter the electrophysiological properties of hERG channels in HEK293 cells.The genetics of cardiac arrhythmias.HERG1 channelopathiesProperties of WT and mutant hERG K(+) channels expressed in neonatal mouse cardiomyocytes.Identification of a trafficking determinant localized to the Kv1 potassium channel poreCeramide modulates HERG potassium channel gating by translocation into lipid raftsDual variation in SCN5A and CACNB2b underlies the development of cardiac conduction disease without Brugada syndrome.Elevated heart rate triggers action potential alternans and sudden death. translational study of a homozygous KCNH2 mutation.Trafficking-deficient G572R-hERG and E637K-hERG activate stress and clearance pathways in endoplasmic reticulum.Traffic jam: a compendium of human diseases that affect intracellular transport processes.Alternative splicing and polyadenylation contribute to the generation of hERG1 C-terminal isoformsNovel characteristics of a trafficking-defective G572R-hERG channel linked to hereditary long QT syndrome.
P2860
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P2860
HERG channel dysfunction in human long QT syndrome. Intracellular transport and functional defects.
description
1998 nî lūn-bûn
@nan
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
1998年论文
@zh
1998年论文
@zh-cn
name
HERG channel dysfunction in hu ...... nsport and functional defects.
@en
type
label
HERG channel dysfunction in hu ...... nsport and functional defects.
@en
prefLabel
HERG channel dysfunction in hu ...... nsport and functional defects.
@en
P2093
P356
P1476
HERG channel dysfunction in hu ...... nsport and functional defects.
@en
P2093
P304
21061-21066
P356
10.1074/JBC.273.33.21061
P407
P577
1998-08-01T00:00:00Z