Article
Modulation of the Ca(2+) permeability of human endplate acetylcholine receptor-channel.
Cell calcium - 1 Apr 2011
Piccari Vanessa, Deflorio Cristina, Bigi Rachele, Grassi Francesca, Fucile Sergio
Abstract excerpt
In slow-channel congenital myasthenic syndrome, point mutations of the endplate acetylcholine receptor (AChR) prolong channel openings, leading to excessive Ca(2+) entry with ensuing endplate degeneration and myasthenic symptoms. The Ca(2+) permeability of the human endplate AChR-channel is quite high, and is further increased by two slow-channel mutations in its ɛ subunit, worsening the pathological cascade. To...
Topics
- Albuterol
- Amino Acid Substitution
- Animals
- Apoptosis
- Calcium
- Cell Line
- Humans
- Motor Endplate
- Mutation
- Myasthenic Syndromes, Congenital
- Patch-Clamp Techniques