Neuromuscular Junction Protection for the Potential Treatment of Amyotrophic Lateral Sclerosis
Figure 2
Schematic detailing NMJ degeneration and treatment. (a) A healthy, functioning NMJ, including TSCs and Ach receptors, is a vital point of communication between MNs in the spinal cord and muscle. (b) In ALS, NMJs begin to degenerate due to a number of pathologies, including disrupted axonal transport and irregular mitochondrial metabolism. NMJ degeneration occurs long before MN degeneration in the spinal cord, preceding clinical symptoms. (c) Ex vivo delivery of GDNF to NMJ via hMSC-GDNF may help to rescue TSCs from degenerative processes, thereby, delaying or preventing degeneration of the NMJ as a whole.
