IndraLab

Statements

KCNH2 inhibits Kr. 20 / 20
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"Mutations in KCNH2 (the gene which encodes I Kr) cause a decrease in the magnitude of I Kr and are associated with Long QT syndrome [XREF_BIBR, XREF_BIBR]."
33604593
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"Reduction of I Kr induced by mutations in hERG or drug block slows repolarization, causing long QT syndrome and sudden cardiac death."
16769794
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"Because hERG channels conduct cardiac I Kr ( 37 , 38 ), a repolarizing current in the heart ( 39 ), hERG blockers can prolong the ventricular action potential and the QT interval on the surface electr[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
31669064
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"A Per-Arnt-Sim (PAS) domain in the hERG1 N-terminus reduces I Kr by slowing channel activation and promoting inactivation."
39786967
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"The widely accepted ionic mechanism of most drug-induced QTc prolongation leading to TdP is direct blockade of Kv11.1 channel (encoded by KCNH2 ) leading to reduced I Kr current ( Katchman et al., 200[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
29698683
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"The potencies (IC 50 values) for E209 to inhibit the cardiac I Kr (hERG), Cav 1.2 and Nav 1.5 channels were determined in an electrophysiology based assays using IonWorks HT (CHO cells) XREF_BIBR."
28537265
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"In SQT1, a gain-of-function mutation in KCNH2 impairs the voltage dependent inactivation of I Kr."
26425105
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"This result indicates that a mutation in hERG not only can disrupt I Kr but can worsen I Ks function and superimpose to cause a synergistic lesion to the defective I Ks encoded with a mutant KCNQ1, leading to further prolongation of APD and the QT interval."
29449639
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"The authors showed that the R311C mutation specifically disables the posttranscriptional activity of TBX20 over KCNH2, which decreases the I Kr and prolongs the AP, therefore, identifying TBX20 as an LQT2 modifying gene [XREF_BIBR]."
28573431
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"We have previously shown that the G1681A mutation in KCNH2 disrupts trafficking of the I Kr / HERG channel to the cell membrane [XREF_BIBR]."
28992755
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"Three predominant types, e.g., LQT type 1 (loss-of-function mutations in KCNQ1 causing a reduction of I Ks ), type 2 (loss-of-function mutations in KCNH2 causing a reduction of I Kr ), and type 3 (gai[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
27210307
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"Mutations in hERG channels reduce I Kr to cause congenital long-QT syndrome type 2, mostly by decreasing surface membrane expression of trafficking deficient channels."
25497881
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"The effects of non hERG channels are thought to enhance or mitigate the torsadogenic effects of I Kr block."
29184497
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"18, 19 Wolpert et al, prove that mutations in KCNH2 reduce the affinity of sotalol in I Kr unlike quinidine."
33335610
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"XREF_BIBR Block of hERG channels reduces the key repolarizing current, I Kr, to cause QT interval lengthening on EKG to potentially induce the life threatening cardiac arrhythmia torsades de pointes."
21131594
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"A reduction in Kv11.1 mediated I Kr current causes LQT2 [ 9 ]."
32387251
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"These findings indicate that heterotetrameric HERG channels containing HERG1b-R25W impair the generation of I Kr."
23571586
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"The hERG channel blocker E-4031 is known to block the rapid delayed rectifier K + channel (I Kr), thereby decreasing K + efflux and increasing APD."
30279735
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"To lengthen atrial refractory period, Amit et al. assessed gene transfer of a KCNH2 mutation (G628S) known to block the I Kr current with dominant negative effects."
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"Using cell biology and electrophysiologic techniques, we found that hypoxic culture of hERG expressing human embryonic kidney (HEK) cells and neonatal rat cardiomyocytes reduced hERG current / I Kr and mature ERG channel expression with a concomitant increase in calpain expression."
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