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August 1, 1998Journal of Biological ChemistryOpen Access

HERG Channel Dysfunction in Human Long QT Syndrome

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Population

HEK 293 cells with stable and transient expression of wild type HERG and LQT-2 mutations

Comparison

LQT-2 mutations vs Wild type HERG

Design

Preclinical

Authors

ZZZhengfeng ZhouQGQiuming GongMEMiles L. Epstein

Discussion

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Overview

Distinct HERG mutation mechanisms in LQT2 extend mechanistic insight; hypothesis-generating from animal data, clinical translation remains open.

Structured PICO

P
Population
HEK 293 cells with stable and transient expression of wild type HERG and LQT-2 mutations
I
Intervention
LQT-2 mutations (Y611H, V822M, I593R, G628S, T474I)
C
Comparator
Wild type HERG
O
Outcome
HERG channel function, processing, and gating properties measured via electrophysiological, biochemical, and immunohistochemical methodssurrogate

The loss of HERG channel function in LQT-2 syndrome is driven by multiple distinct molecular mechanisms, including abnormal processing, generation of nonfunctional channels, and altered gating.

Cite This Study

Zhou et al. (1998) studied this question.

synapsesocial.com/papers/69f14b792811130d0cde21bahttps://doi.org/10.1074/jbc.273.33.21061
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