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Article

HERG trafficking and pharmacological rescue of LQTS-2 mutant channels.

Handbook of experimental pharmacology - 1 Jan 2006

Robertson G A, January C T

Abstract excerpt

The human ether-a-go-go-related gene (hERG) encodes an ion channel subunit underlying IKr, a potassium current required for the normal repolarization of ventricular cells in the human heart. Mutations in hERG cause long QT syndrome (LQTS) by disrupting IKr, increasing cardiac excitability and, in some cases, triggering catastrophic torsades de pointes arrhythmias and sudden death. More than 200 putative...

Topics

Animals
ERG1 Potassium Channel
Ether-A-Go-Go Potassium Channels
Humans
Long QT Syndrome
Mutation
Protein Transport

Source

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Identifiers

PMID: 16610352
DOI: 10.1007/3-540-29715-4_14

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HERG trafficking and pharmacological rescue of LQTS-2 mutant channels.PMID 16610352Read ↗

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Pharmacological rescue of human K(+) channel long-QT2 mutations: human ether-a-go-go-related gene rescue without block.Semantic proximity Read ↗

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

The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations.Semantic proximity Read ↗

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Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects.Semantic proximity Read ↗

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The mutation L69P in the PAS domain of the hERG potassium channel results in LQTS by trafficking deficiency.Semantic proximity Read ↗

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