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Article

Hsp40 chaperones promote degradation of the HERG potassium channel.

The Journal of biological chemistry - 29 Jan 2010

Walker Valerie E, Wong Michael J H, Atanasiu Roxana, Hantouche Christine, Young Jason C, Shrier Alvin

Abstract excerpt

Loss of function mutations in the hERG (human ether-a-go-go related gene or KCNH2) potassium channel underlie the proarrhythmic cardiac long QT syndrome type 2. Most often this is a consequence of defective trafficking of hERG mutants to the cell surface, with channel retention and degradation at the endoplasmic reticulum. Here, we identify the Hsp40 type 1 chaperones DJA1 (DNAJA1/Hdj2) and DJA2 (DNAJA2) as key...

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Source

Open in PubMed Open full text

Identifiers

PMID: 19940115
DOI: 10.1074/jbc.M109.024000
PMCID: PMC2823420

Genes

DNAJA1
DNAJA2
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.

Related research

Semantic proximity does not establish scientific evidence.

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Hsp40 chaperones promote degradation of the HERG potassium channel.PMID 19940115Read ↗

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Regulation of the human ether-a-go-go-related gene (hERG) potassium channel by Nedd4 family interacting proteins (Ndfips).Semantic proximity Read ↗

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ERdj3 is an endoplasmic reticulum degradation factor for mutant glucocerebrosidase variants linked to Gaucher's disease.Semantic proximity Read ↗

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

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