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Article

Interaction with GM130 during HERG ion channel trafficking. Disruption by type 2 congenital long QT syndrome mutations. Human Ether-à-go-go-Related Gene.

The Journal of biological chemistry - 6 Dec 2002

Roti Elon C Roti, Myers Cena D, Ayers Rebecca A, Boatman Dorothy E, Delfosse Samantha A, Chan Edward K L, Ackerman Michael J, January Craig T, Robertson Gail A

Abstract excerpt

Many mutations in the Human Ether-à-go-go-Related Gene (HERG) cause type 2 congenital long QT syndrome (LQT2) by disrupting trafficking of the HERG-encoded potassium channel. Beyond observations that some mutations trap channels in the endoplasmic reticulum, little is known about how trafficking fails. Even less is known about what checkpoints are encountered in normal trafficking. To identify protein partners...

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Identifiers

PMID: 12270925
DOI: 10.1074/jbc.M206638200

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Interaction with GM130 during HERG ion channel trafficking. Disruption by type 2 congenital long QT syndrome mutations. Human Ether-à-go-go-Related Gene.PMID 12270925Read ↗

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

Defective human Ether-à-go-go-related gene trafficking linked to an endoplasmic reticulum retention signal in the C terminus.Semantic proximity Read ↗

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

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

GOLGA2, encoding a master regulator of golgi apparatus, is mutated in a patient with a neuromuscular disorder.Semantic proximity Read ↗

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

hERG 1a LQT2 C-terminus truncation mutants display hERG 1b-dependent dominant negative mechanisms.Semantic proximity Read ↗

Identification of the cyclic-nucleotide-binding domain as a conserved determinant of ion-channel cell-surface localization.Semantic proximity Read ↗

Mechanisms of pharmacological rescue of trafficking-defective hERG mutant channels in human long QT syndrome.Semantic proximity Read ↗

Development of a High-Throughput Flow Cytometry Assay to Monitor Defective Trafficking and Rescue of Long QT2 Mutant hERG ChannelsSemantic proximity Read ↗

Characterization of S818L mutation in HERG C-terminus in LQT2. Modification of activation-deactivation gating properties.Semantic proximity Read ↗

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