title: Potassium channel blockers and impulse propagation in murine motor endplate disease.
abstract: An electrophysiologic study has been performed on motor nerves of mice affected with hereditary "motor endplate disease" (MED). Bath application of potassium channel blockers, such as tetraethylammonium and 3,4-diaminopyridine, which are almost without effect on the monophasic compound action potential of normal nerves, considerably enhanced the action potential duration in nerves from mutant mice. Furthermore, external current recordings from motor endings revealed an absence of the K-dependent waveform component in MED mice, which indicates a similar K current intensity in the terminal part of the endings and in the heminode. These observations suggest that in the mutant, unlike in normal mice, K channels play a role in action potential electrogenesis. Possible relationships with paranodal dysmyelination are discussed.
mesh_terms: D015761:4-Aminopyridine / Q000031:analogs & derivatives*; D000200:Action Potentials / Q000187:drug effects; D000077770:Amifampridine; D000631:Aminopyridines / Q000494:pharmacology; D000818:Animals; D066298:In Vitro Techniques; D007473:Ion Channels / Q000187:drug effects / Q000378:metabolism*; D051379:Mice; D008809:Mice, Inbred C3H; D009045:Motor Endplate / Q000187:drug effects / Q000378:metabolism*; D009468:Neuromuscular Diseases / Q000188:drug therapy / Q000378:metabolism*; D009469:Neuromuscular Junction / Q000378:metabolism*; D011188:Potassium / Q000378:metabolism*; D019789:Tetraethylammonium; D013757:Tetraethylammonium Compounds / Q000494:pharmacology
chemical_list: D000631:Aminopyridines; D007473:Ion Channels; D013757:Tetraethylammonium Compounds; D019789:Tetraethylammonium; D015761:4-Aminopyridine; D000077770:Amifampridine; D011188:Potassium