Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel
about
Translational toxicology and rescue strategies of the hERG channel dysfunction: biochemical and molecular mechanistic aspectsStructural properties of PAS domains from the KCNH potassium channelsSpinocerebellar ataxia type 19/22 mutations alter heterocomplex Kv4.3 channel function and gating in a dominant mannerCombined gating and trafficking defect in Kv11.1 manifests as a malignant long QT syndrome phenotype in a large Danish p.F29L founder family.Role of the cytoplasmic N-terminal Cap and Per-Arnt-Sim (PAS) domain in trafficking and stabilization of Kv11.1 channels.C-terminal β9-strand of the cyclic nucleotide-binding homology domain stabilizes activated states of Kv11.1 channels.A TRPV4 channel C-terminal folding recognition domain critical for trafficking and function.Mechanistic basis for type 2 long QT syndrome caused by KCNH2 mutations that disrupt conserved arginine residues in the voltage sensor.Enhancement of hERG channel activity by scFv antibody fragments targeted to the PAS domainLQT2 nonsense mutations generate trafficking defective NH2-terminally truncated channels by the reinitiation of translation.Ubiquitination-dependent quality control of hERG K+ channel with acquired and inherited conformational defect at the plasma membrane.The role of the cytosolic HSP70 chaperone system in diseases caused by misfolding and aberrant trafficking of ion channelsThe enigmatic cytoplasmic regions of KCNH channels.Getting to the heart of hERG K(+) channel gating.hERG quality control and the long QT syndrome.Screening for Non-Pore-Binding Modulators of EAG K+ Channels.Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome.Trafficking defects in PAS domain mutant Kv11.1 channels: roles of reduced domain stability and altered domain-domain interactions.Development of a High-Throughput Flow Cytometry Assay to Monitor Defective Trafficking and Rescue of Long QT2 Mutant hERG Channels.
P2860
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P2860
Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel
description
2012 nî lūn-bûn
@nan
2012 թուականին հրատարակուած գիտական յօդուած
@hyw
2012 թվականին հրատարակված գիտական հոդված
@hy
2012年の論文
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2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
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name
Changes in channel trafficking ...... PAS domain of the HERG channel
@ast
Changes in channel trafficking ...... PAS domain of the HERG channel
@en
Changes in channel trafficking ...... PAS domain of the HERG channel
@nl
type
label
Changes in channel trafficking ...... PAS domain of the HERG channel
@ast
Changes in channel trafficking ...... PAS domain of the HERG channel
@en
Changes in channel trafficking ...... PAS domain of the HERG channel
@nl
prefLabel
Changes in channel trafficking ...... PAS domain of the HERG channel
@ast
Changes in channel trafficking ...... PAS domain of the HERG channel
@en
Changes in channel trafficking ...... PAS domain of the HERG channel
@nl
P2860
P50
P1433
P1476
Changes in channel trafficking ...... PAS domain of the HERG channel
@en
P2093
Ricardo Carvalho
P2860
P304
P356
10.1371/JOURNAL.PONE.0032654
P407
P577
2012-03-02T00:00:00Z