Defects in neuromuscular junction remodelling in the Smn2B/− mouse model of spinal muscular atrophy
Spinal muscular atrophy (SMA) is a devastating childhood motor neuron disease caused by mutations and deletions within the survival motor neuron 1 (SMN1) gene. Although other tissues may be involved, motor neurons remain primary pathological targets, with loss of neuromuscular junctions (NMJs) repre...
Main Authors: | , , , |
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Format: | Article |
Language: | English |
Published: |
Elsevier
2013-01-01
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Series: | Neurobiology of Disease |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0969996112003063 |