Reversal of paralysis in nerve-muscle preparations isolated from animals with hereditary motor endplate disease

Br J Pharmacol. 1985 Jan;84(1):6-8.

Abstract

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.

MeSH terms

  • 4-Aminopyridine
  • Aminopyridines / therapeutic use*
  • Animals
  • Mice
  • Motor Endplate / physiopathology*
  • Neuromuscular Diseases / complications
  • Neuromuscular Diseases / genetics*
  • Neuromuscular Diseases / physiopathology
  • Neuromuscular Junction / physiopathology*
  • Paralysis / drug therapy*
  • Paralysis / etiology
  • Paralysis / physiopathology

Substances

  • Aminopyridines
  • 4-Aminopyridine