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February 1, 2002Journal of Biological ChemistryOpen Access

The Binding Site for Channel Blockers That Rescue Misprocessed Human Long QT Syndrome Type 2 ether-a-gogo-related Gene (HERG) Mutations

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Population

In vitro model of human ether-a-gogo-related gene K channels with Long QT Syndrome Type 2 mutations

Design

Preclinical

Authors

EFEckhard FickerMetroHealthSZShu-Xia ZhaoDalian University of TechnologyCOCarlos A. Obejero‐PazPalo Alto University

Discussion

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Implication

May guide non-blocking chaperone design for LQT2; hypothesis-generating in animal models and should not yet change practice.

Structured PICO

P
Population
In vitro model of human ether-a-gogo-related gene (HERG) K(+) channels with Long QT Syndrome Type 2 (LQT2) mutations (HERG G601S, HERG F805C, HERG R823W)
I
Intervention
HERG channel blockers acting as pharmacological chaperones
O
Outcome
Cell surface expression and rescue of trafficking-deficient mutant HERG channelssurrogate

Pharmacological chaperones (HERG channel blockers) can rescue trafficking-deficient HERG G601S mutations by binding to the inner cavity, providing a structural basis for designing non-blocking chaperones to treat LQT2.

Cite This Study

Ficker et al. (2002) studied this question.

synapsesocial.com/papers/6a0f9dcad03631df9ce9ccddhttps://doi.org/10.1074/jbc.m107345200
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Interaction of Tetraethylammonium Ion Derivatives with the Potassium Channels of Giant Axons1971 · 937 citations
  2. 2Correction of Defective Protein Trafficking of a Mutant HERG Potassium Channel in Human Long QT Syndrome1999 · 300 citations
  3. 3Novel Mechanism Associated With an Inherited Cardiac Arrhythmia1999 · 193 citations
  4. 4Analysis of the Cyclic Nucleotide Binding Domain of the HERG Potassium Channel and Interactions with KCNE22001 · 89 citations
  5. 5Molecular Physiology and Pharmacology ofHERG1996 · 212 citations