Despite being an early event in ALS, it remains unclear whether the denervation of neuromuscular junctions (NMJ) is simply the first manifestation of a globally degenerating motor neuron. Using in vivo imaging of single axons and their NMJs over a three-month period, we identify that single motor-units are dismantled asynchronously in SOD1G37R mice. We reveal that weeks prior to complete axonal degeneration, the dismantling of axonal branches is accompanied by contemporaneous new axonal sprouting resulting in synapse formation onto nearby NMJs. Denervation events tend to propagate from the first lost NMJ, consistent with a contribution of neuromuscular factors extrinsic to motor neurons, with distal branches being more susceptible. These results show that NMJ denervation in ALS is a complex and dynamic process of continuous denervation and new innervation rather than a manifestation of sudden global motor neuron degeneration.
Pubmed ID: 30320556 RIS Download
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View all literature mentionsThis unknown targets Neurofilament M Antibody
View all literature mentionsThis unknown targets Mouse Bovine myosin heavy chain slow alpha- and beta-
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View all literature mentionsThis monoclonal targets Myosin heavy chain (all but 2X)
View all literature mentionsThis monoclonal targets Myosin Heavy Chain Type IIB
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View all literature mentionsThis monoclonal targets Myosin heavy chain, fast, 2X
View all literature mentionsThis unknown targets Mouse IgG, Fc? Subclass 1 Specific
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View all literature mentionsThis unknown targets Rabbit IgG (H+L)
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