Article
Reconstitution of defective protein trafficking rescues Long-QT syndrome in zebrafish.
Biochemical and biophysical research communications - 6 May 2011
Meder Benjamin, Scholz Eberhard P, Hassel David, Wolff Christoph, Just Steffen, Berger Ina M, Patzel Eva, Karle Christoph, Katus Hugo A, Rottbauer Wolfgang
Abstract excerpt
Inherited cardiac arrhythmias are caused by genetic defects in ion channels and associated proteins. Mutations in these channels often do not affect their biophysical properties, but rather interfere with their trafficking to the cell membrane. Accordingly, strategies that could reroute the mutated channels to the membrane should be sufficient to restore the electrical properties of the affected cells, thereby...
Topics
- Animals
- Disease Models, Animal
- Ether-A-Go-Go Potassium Channels
- HEK293 Cells
- Heart Rate
- Humans
- Long QT Syndrome
- Mutation
- Protein Transport
- Ventricular Dysfunction