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Cited by 383

The DOI matches its Crossref record; 32 of the 32 references it cites were found in a registry or an index.

HERG Channel Dysfunction in Human Long QT Syndrome

Open access
Zhou, Zhengfeng, Gong, Qiuming, Epstein, Miles L., January, Craig T.·1998·Journal of Biological Chemistry

Abstract

Mutations in HERG are associated with human chromosome 7-linked congenital long QT (LQT-2) syndrome. We used electrophysiological, biochemical, and immunohistochemical methods to study the molecular mechanisms of HERG channel dysfunction caused by LQT-2 mutations. Wild type HERG and LQT-2 mutations were studied by stable and transient expression in HEK 293 cells. We found that some mutations (Y611H and V822M) caused defects in biosynthetic processing of HERG channels with the protein retained in the endoplasmic reticulum. Other mutations (I593R and G628S) were processed similarly to wild type HERG protein, but these mutations did not produce functional channels. In contrast, the T474I mutation expressed HERG current but with altered gating properties. These findings suggest that the loss of HERG channel function in LQT-2 mutations is caused by multiple mechanisms including abnormal channel processing, the generation of nonfunctional channels, and altered channel gating.

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Metadata

Authors
Zhou, Z., Gong, Q., Epstein, M. L., & January, C. T.
Venue
Journal of Biological Chemistry
Publisher
Elsevier BV
Published
1998-08
Volume
273
Issue
33
Pages
21061-21066
ISSN
0021-9258 (ISSN-L)
OpenAlex
W1963890550
Cited by
383
References
32
FWCI
15.34
Type
journal article
Language
en
Open access
hybrid

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CoChat. (2026). Citation check: HERG Channel Dysfunction in Human Long QT Syndrome. https://app.cochat.ai/papers/10.1074/jbc.273.33.21061
The paper
Zhou, Z., Gong, Q., Epstein, M. L., & January, C. T. (1998). HERG Channel Dysfunction in Human Long QT Syndrome. Journal of Biological Chemistry. https://doi.org/10.1074/jbc.273.33.21061

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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