Article
A global defect in scaling relationship between electrical activity and availability of muscle sodium channels in hyperkalemic periodic paralysis.
Pflugers Archiv : European journal of physiology - 1 Jul 1999
Melamed-Frank M, Marom S
Abstract excerpt
Hyperkalemic periodic paralysis (HyperPP) is a hereditary disorder characterized by alternate episodic attacks of muscle weakness and muscle myotonia. The most common mutation associated with HyperPP is a T704M substitution in the skeletal-muscle sodium channel. This mutation increases sodium persistent currents, alters voltage dependence of activation and impairs slow inactivation. The present study shows...
Topics
- Cell Line
- Electrophysiology
- Humans
- Ion Channel Gating
- Kinetics
- Muscle, Skeletal
- Mutation
- Paralyses, Familial Periodic
- Plasmids
- Sodium Channels