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Int J Mol Sci. 2017 Oct 3;18(10). pii: E2092. doi: 10.3390/ijms18102092.

Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis.

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Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro 12, 56127 Pisa, Italy.
Department of Medical Biotechnology and Translational Medicine, Università degli Studi di Milano-Via Vanvitelli 32, 20129 Milano, Italy.


Neuromuscular junction assembly and plasticity during embryonic, postnatal, and adult life are tightly regulated by the continuous cross-talk among motor nerve endings, muscle fibers, and glial cells. Altered communications among these components is thought to be responsible for the physiological age-related changes at this synapse and possibly for its destruction in pathological states. Neuromuscular junction dismantling plays a crucial role in the onset of Amyotrophic Lateral Sclerosis (ALS). ALS is characterized by the degeneration and death of motor neurons leading to skeletal muscle denervation, atrophy and, most often, death of the patient within five years from diagnosis. ALS is a non-cell autonomous disease as, besides motor neuron degeneration, glial cells, and possibly muscle fibers, play a role in its onset and progression. Here, we will review the recent literature regarding the mechanisms leading to neuromuscular junction disassembly and muscle denervation focusing on the role of the three players of this peripheral tripartite synapse.


Amyotrophic Lateral Sclerosis; neuromuscular junction; tripartite synapse

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