Neuromuscular junction destruction during amyotrophic lateral sclerosis: insights from transgenic models
L Dupuis, JP Loeffler - Current opinion in pharmacology, 2009 - Elsevier
Amyotrophic lateral sclerosis (ALS) represents the major adult-onset motor neuron disease.
Analyses of ALS animal models have shown that motor neuron death starts with
neuromuscular junction (NMJ) destruction and distal axonal degeneration. Most importantly,
motor neuron death results from pathological events occurring outside the motor neuron,
especially in glial cells and skeletal muscle and, surprisingly, is associated with pathological
defects outside the motor system. In particular, ALS pathogenesis includes systemic defects …
Analyses of ALS animal models have shown that motor neuron death starts with
neuromuscular junction (NMJ) destruction and distal axonal degeneration. Most importantly,
motor neuron death results from pathological events occurring outside the motor neuron,
especially in glial cells and skeletal muscle and, surprisingly, is associated with pathological
defects outside the motor system. In particular, ALS pathogenesis includes systemic defects …