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Article

Trafficking-deficient hERG K⁺ channels linked to long QT syndrome are regulated by a microtubule-dependent quality control compartment in the ER.

American journal of physiology. Cell physiology - 1 Jul 2011

Smith Jennifer L, McBride Christie M, Nataraj Parvathi S, Bartos Daniel C, January Craig T, Delisle Brian P

Abstract excerpt

The human ether-a-go-go related gene (hERG) encodes the voltage-gated K(+) channel that underlies the rapidly activating delayed-rectifier current in cardiac myocytes. hERG is synthesized in the endoplasmic reticulum (ER) as an "immature" N-linked glycoprotein and is terminally glycosylated in the Golgi apparatus. Most hERG missense mutations linked to long QT syndrome type 2 (LQT2) reduce the terminal...

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Identifiers

PMID: 21490315
DOI: 10.1152/ajpcell.00494.2010
PMCID: PMC3129823

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

Related research

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Trafficking-deficient hERG K⁺ channels linked to long QT syndrome are regulated by a microtubule-dependent quality control compartment in the ER.PMID 21490315Read ↗

Defective protein trafficking in hERG-associated hereditary long QT syndrome (LQT2): molecular mechanisms and restoration of intracellular protein processing.Semantic proximity Read ↗

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Role of the cytosolic chaperones Hsp70 and Hsp90 in maturation of the cardiac potassium channel HERG.Semantic proximity Read ↗

Molecular pathogenesis of long QT syndrome type 2Semantic proximity Read ↗

Novel mechanism associated with an inherited cardiac arrhythmia: defective protein trafficking by the mutant HERG (G601S) potassium channel.Semantic proximity Read ↗

Long QT syndrome-associated I593R mutation in HERG potassium channel activates ER stress pathways.Semantic proximity Read ↗

HERG trafficking and pharmacological rescue of LQTS-2 mutant channels.Semantic proximity Read ↗

Identification of a COOH-terminal segment involved in maturation and stability of human ether-a-go-go-related gene potassium channels.Semantic proximity Read ↗

Pharmacological rescue of hERG currents carried out by G604S and wide type hERG co-expression.Semantic proximity Read ↗

Identification and functional characterization of the human ether-a-go-go-related gene Q738X mutant associated with hereditary long QT syndrome type 2.Semantic proximity Read ↗

N-linked glycosylation sites determine HERG channel surface membrane expression.Semantic proximity Read ↗

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