[HTML][HTML] Low dystrophin levels are insufficient to normalize the neuromuscular synaptic abnormalities of mdx mice

EM van der Pijl, M van Putten, EH Niks… - Neuromuscular …, 2018 - Elsevier
Dystrophin is a sub-sarcolemmal component of skeletal muscle fibres and is enriched at the
postsynaptic membrane of the neuromuscular junction (NMJ). In the mdx mouse, dystrophin
absence not only causes muscle damage but also mild synaptic dysfunctions and clear
morphological aberrations at NMJs. In particular, reduction of postsynaptic sensitivity for the
neurotransmitter acetylcholine and extra exhaustion of presynaptic acetylcholine release
during intense synaptic activity exists. Current experimental therapeutic approaches in …