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Article

Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism.

Circulation - 24 Jan 2006

Anderson Corey L, Delisle Brian P, Anson Blake D, Kilby Jennifer A, Will Melissa L, Tester David J, Gong Qiuming, Zhou Zhengfeng, Ackerman Michael J, January Craig T

Abstract excerpt

BACKGROUND: The KCNH2 or human ether-a-go-go related gene (hERG) encodes the Kv11.1 alpha-subunit of the rapidly activating delayed rectifier K+ current (IKr) in the heart. Type 2 congenital long-QT syndrome (LQT2) results from KCNH2 mutations that cause loss of Kv11.1 channel function. Several mechanisms have been identified, including disruption of Kv11.1 channel synthesis (class 1), protein trafficking (class...

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Source

Identifiers

PMID: 16432067
DOI: 10.1161/CIRCULATIONAHA.105.570200

Genes

KCNH2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism.PMID 16432067Read ↗

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High-throughput discovery of trafficking-deficient variants in the cardiac potassium channel <i>KCNH2</i> : Deep mutational scan of <i>KCNH2</i> traffickingSemantic proximity Read ↗

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