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September 1, 2003Journal of Biological ChemistryOpen Access

Thapsigargin Selectively Rescues the Trafficking Defective LQT2 Channels G601S and F805C

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Why the study?

Does thapsigargin rescue the surface expression of trafficking defective LQT2 mutations without blocking HERG current in HEK293 cells?

Population

Human embryonic kidney 293 cell lines stably expressing WT HERG and the trafficking defective LQT2 mutations…

Comparison

Thapsigargin (1 μm) vs E4031, other SERCA inhibitors, or vehicle

Design

Preclinical

Authors

BDBrian P. DelisleElectrophysiologyCACorey L. AndersonUniversity of Wisconsin–MadisonRBRavi C. BalijepalliNational Heart Lung and Blood Institute

Discussion

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Implication

Hypothesis-generating for non-blocking rescue in select LQT2 mutations; leaves open translation beyond HEK293 cells.

Structured PICO

Does thapsigargin rescue the surface expression of trafficking defective LQT2 mutations without blocking HERG current in HEK293 cells?

P
Population
Human embryonic kidney 293 (HEK293) cell lines stably expressing WT HERG and the trafficking defective LQT2 mutations N470D, G601S, and F805C
I
Intervention
Thapsigargin (1 μm)
C
Comparator
E4031, other SERCA inhibitors (DBHQ, cyclopiazonic acid), or vehicle (0.1% Me2SO)
O
Outcome
Surface expression of mutant HERG channels and HERG current blocksurrogate

Thapsigargin selectively rescues the surface expression of specific trafficking-defective LQT2 mutations (G601S and F805C) without blocking the HERG channel current, demonstrating proof-of-concept for non-blocking pharmacological rescue.

Cite This Study

Delisle et al. (2003) studied this question.

synapsesocial.com/papers/6a0f9dcad03631df9ce9ccdahttps://doi.org/10.1074/jbc.m305787200
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Also Consider

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

  1. 1The Binding Site for Channel Blockers That Rescue Misprocessed Human Long QT Syndrome Type 2 ether-a-gogo-related Gene (HERG) Mutations2002 · 184 citations
  2. 2Correction of Defective Protein Trafficking of a Mutant HERG Potassium Channel in Human Long QT Syndrome1999 · 300 citations
  3. 3A Novel Mutation (T65P) in the PAS Domain of the Human Potassium Channel HERG Results in the Long QT Syndrome by Trafficking Deficiency2002 · 95 citations
  4. 4Novel Mechanism Associated With an Inherited Cardiac Arrhythmia1999 · 193 citations
  5. 5Long QT Syndrome:2000 · 113 citations