Article
Trafficking defects in PAS domain mutant Kv11.1 channels: roles of reduced domain stability and altered domain-domain interactions.
The Biochemical journal - 15 Aug 2013
Ke Ying, Ng Chai Ann, Hunter Mark J, Mann Stefan A, Heide Juliane, Hill Adam P, Vandenberg Jamie I
Abstract excerpt
Loss of Kv11.1 potassium channel function is the underlying cause of pathology in long-QT syndrome type 2, one of the commonest causes of sudden cardiac death in the young. Previous studies have identified the cytosolic PAS (Per/Arnt/Sim) domain as a hotspot for mutations that cause Kv11.1 trafficking defects. To investigate the underlying basis of this observation, we have quantified the effect of mutants on...
Topics
- Animals
- CHO Cells
- Cricetinae
- Cricetulus
- ERG1 Potassium Channel
- Ether-A-Go-Go Potassium Channels
- Female
- HEK293 Cells
- Humans
- Mutation
- Protein Binding
- Protein Serine-Threonine Kinases
- Protein Transport
- Xenopus laevis