Reversal of paralysis in nerve-muscle preparations isolated from animals with hereditary motor endplate disease.
Motor endplate disease (med) in the mouse is an hereditary disorder of the skeletal neuromuscular system. Affected animals suffer a 'functional denervation' of skeletal muscle (Duchen & Stefani, 1971). Muscle fibres do not respond to indirect excitation, but motor nerve terminals release transmitter spontaneously. Spontaneous transmitter release can be enhanced by raising [K+]o or by exposing muscles to red-back spider venom and functional transmission following indirect stimulation may be restored by 4-aminopyridine.
Harris
J B
JB
Pollard
S L
SL
D015761:4-Aminopyridine; D000631:Aminopyridines / Q000627:therapeutic use*; D000818:Animals; D051379:Mice; D009045:Motor Endplate / Q000503:physiopathology*; D009468:Neuromuscular Diseases / Q000150:complications / Q000235:genetics* / Q000503:physiopathology; D009469:Neuromuscular Junction / Q000503:physiopathology*; D010243:Paralysis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology
D000631:Aminopyridines; D015761:4-Aminopyridine