Ontology highlight
ABSTRACT:
SUBMITTER: Walker VE
PROVIDER: S-EPMC2823420 | biostudies-literature | 2010 Jan
REPOSITORIES: biostudies-literature
The Journal of biological chemistry 20091125 5
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 modulators of hERG degradation. Overexpression of the DJAs reduces hERG trafficking e ...[more]