Premium
Neuromuscular transmission in the mdx mouse
Author(s) -
Nagel Alexandre,
LehmannHorn Frank,
Engel Andrew G.
Publication year - 1990
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880130813
Subject(s) - neuromuscular transmission , duchenne muscular dystrophy , mdx mouse , neuromuscular disease , medicine , neuromuscular junction , physical medicine and rehabilitation , neuroscience , biology , dystrophin , disease
The mdx mouse is an animal model for human Duchenne dystrophy. In both disorders, the muscle fiber plasma membrane is rendered selectively vulnerable by dystrophin deficiency. In both disorders there are also ultrastructural abnormalities involving the postsynaptic membrane of the neuromuscular junction. The object of this electrophysiologic study was to determine whether the observed ultrastructural abnormalities at the mdx neuromuscular junction are associated with an abnormality of neuromuscular transmission. In comparison with age‐matched control mice, the mdx mice show an abnormal, age‐dependent decrease of the amplitude of the miniature end‐plate potential and aconcomitant increase in the quantal content of the end‐plate potential. Consequently, the safety margin of neuromuscular transmission is not impaired.