TY - JOUR
AU1 - Verma, Sagar
AU2 - Khurana, Shiffali
AU3 - Vats, Abhishek
AU4 - Sahu, Bandana
AU5 - Ganguly, Nirmal Kumar
AU6 - Chakraborti, Pradip
AU7 - Gourie-Devi, Mandaville
AU8 - Taneja, Vibha
AB - Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier studies have shown that motor neuron degeneration begins in motor cortex and descends to the neuromuscular junction (NMJ) in a dying forward fashion. However, accumulating evidences support that ALS is a distal axonopathy where early pathological changes occur at the NMJ, prior to onset of clinical symptoms and propagates towards the motor neuron cell body supporting “dying back” hypothesis. Despite several evidences, series of events triggering NMJ disassembly in ALS are still obscure. Neuromuscular junction is a specialized tripartite chemical synapse which involves a well-coordinated communication among the presynaptic motor neuron, postsynaptic skeletal muscle, and terminal Schwann cells. This review provides comprehensive insight into the role of NMJ in ALS pathogenesis. We have emphasized the molecular alterations in cellular components of NMJ leading to loss of effective neuromuscular transmission in ALS. Further, we provide a preview into research involved in exploring NMJ as potential target for designing effective therapies for ALS. 
TI - Neuromuscular Junction Dysfunction in Amyotrophic Lateral Sclerosis
JF - Molecular Neurobiology
DO - 10.1007/s12035-021-02658-6
DA - 2022-03-01
UR - https://www.deepdyve.com/lp/springer-journals/neuromuscular-junction-dysfunction-in-amyotrophic-lateral-sclerosis-HnX4lZ0qGA
SP - 1502
EP - 1527
VL - 59
IS - 3
DP - DeepDyve
ER -