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Article

Co-chaperone FKBP38 promotes HERG trafficking.

The Journal of biological chemistry - 10 Aug 2007

Walker Valerie E, Atanasiu Roxana, Lam Hung, Shrier Alvin

Abstract excerpt

The Long QT Syndrome is a cardiac disorder associated with ventricular arrhythmias that can lead to syncope and sudden death. One prominent form of the Long QT syndrome has been linked to mutations in the HERG gene (KCNH2) that encodes the voltage-dependent delayed rectifier potassium channel (I(Kr)). In order to search for HERG-interacting proteins important for HERG maturation and trafficking, we conducted a...

Topics

Cell Line
Cell Membrane
Cytosol
ERG1 Potassium Channel
Ether-A-Go-Go Potassium Channels
HSC70 Heat-Shock Proteins
HSP90 Heat-Shock Proteins
Humans
Mass Spectrometry
Models, Biological
Molecular Chaperones

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Mutation

Protein Transport

RNA, Small Interfering

Tacrolimus Binding Proteins

Transfection

Source

Identifiers

PMID: 17569659
DOI: 10.1074/jbc.M701006200

Genes

FKBP8
KCNH2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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Co-chaperone FKBP38 promotes HERG trafficking.PMID 17569659Read ↗

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

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

Trafficking defect and proteasomal degradation contribute to the phenotype of a novel KCNH2 long QT syndrome mutation.Semantic proximity Read ↗

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

Novel characteristics of a trafficking-defective G572R-hERG channel linked to hereditary long QT syndrome.Semantic proximity Read ↗

A Novel KCNH2 S981fs Mutation Identified by Whole-Exome Sequencing Is Associated with Type 2 Long QT Syndrome.Semantic proximity Read ↗

Partially dominant mutant channel defect corresponding with intermediate LQT2 phenotype.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 ↗

Hsp40 chaperones promote degradation of the HERG potassium channel.Semantic proximity Read ↗

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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 ↗

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