hERG Quality Control at the Plasma Membrane

Pirjo Apaja; Tsukasa  Okiyondea; Herve Barrière; Hung  Lam; Roxana Atanasiu; Brian Foo; Eckhard Ficker; Gergely L. Lukacs; Alvin Shrier

doi:10.1016/j.bpj.2011.11.3683

hERG Quality Control at the Plasma Membrane

Pirjo Apaja, Tsukasa Okiyondea, Herve Barrière, Hung Lam, Roxana Atanasiu, Brian Foo, Eckhard Ficker, Gergely L. Lukacs, Alvin Shrier

Research output: Contribution to journal › Article › peer-review

Abstract

The KCNH2 or human ether-a-go-go related gene (HERG1) encodes the
Kv11.1 protein that is responsible for the rapidly activating delayed rectifier
Kþ current (IKr). Mutations of HERG cause prolongation of cardiac ventricular repolarzation that underlies the long QT syndrome (LQT2) and
associated risk of sudden death. Genetic analyses have identified over 200
LQT2-associated mutations the majority of which impair the exit of
HERG from the endoplasmic reticulum (ER).

Original language	English
Pages (from-to)	677a
Number of pages	1
Journal	Biophysical Journal
Volume	102
Issue number	3 Suppl 1
DOIs	https://doi.org/10.1016/j.bpj.2011.11.3683
Publication status	Published - 31 Jan 2012
Externally published	Yes

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title = "hERG Quality Control at the Plasma Membrane",

abstract = "The KCNH2 or human ether-a-go-go related gene (HERG1) encodes theKv11.1 protein that is responsible for the rapidly activating delayed rectifierK{\th} current (IKr). Mutations of HERG cause prolongation of cardiac ventricular repolarzation that underlies the long QT syndrome (LQT2) andassociated risk of sudden death. Genetic analyses have identified over 200LQT2-associated mutations the majority of which impair the exit ofHERG from the endoplasmic reticulum (ER).",

author = "Pirjo Apaja and Tsukasa Okiyondea and Herve Barri{\`e}re and Hung Lam and Roxana Atanasiu and Brian Foo and Eckhard Ficker and Lukacs, {Gergely L.} and Alvin Shrier",

year = "2012",

month = jan,

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doi = "10.1016/j.bpj.2011.11.3683",

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T1 - hERG Quality Control at the Plasma Membrane

AU - Apaja, Pirjo

AU - Okiyondea, Tsukasa

AU - Barrière, Herve

AU - Lam, Hung

AU - Atanasiu, Roxana

AU - Foo, Brian

AU - Ficker, Eckhard

AU - Lukacs, Gergely L.

AU - Shrier, Alvin

PY - 2012/1/31

Y1 - 2012/1/31

N2 - The KCNH2 or human ether-a-go-go related gene (HERG1) encodes theKv11.1 protein that is responsible for the rapidly activating delayed rectifierKþ current (IKr). Mutations of HERG cause prolongation of cardiac ventricular repolarzation that underlies the long QT syndrome (LQT2) andassociated risk of sudden death. Genetic analyses have identified over 200LQT2-associated mutations the majority of which impair the exit ofHERG from the endoplasmic reticulum (ER).

AB - The KCNH2 or human ether-a-go-go related gene (HERG1) encodes theKv11.1 protein that is responsible for the rapidly activating delayed rectifierKþ current (IKr). Mutations of HERG cause prolongation of cardiac ventricular repolarzation that underlies the long QT syndrome (LQT2) andassociated risk of sudden death. Genetic analyses have identified over 200LQT2-associated mutations the majority of which impair the exit ofHERG from the endoplasmic reticulum (ER).

U2 - 10.1016/j.bpj.2011.11.3683

DO - 10.1016/j.bpj.2011.11.3683

M3 - Article

SN - 0006-3495

VL - 102

SP - 677a

JO - Biophysical Journal

JF - Biophysical Journal

IS - 3 Suppl 1

ER -

hERG Quality Control at the Plasma Membrane

Abstract

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