Neuromuscular junction disorders: Experimental models and pathophysiological mechanisms
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Published:2022-12-30
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ISSN:1689-0035
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Container-title:Acta Neurobiologiae Experimentalis
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language:
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Short-container-title:Acta Neurobiol Exp (Wars)
Author:
Yan Li,Yin Zhao-Xu,Chang Xue-Li,Wang Rui,Zhang Xiu-Min,Guo Jun-Hong
Abstract
Located between skeletal muscle fibers and motoneurons, the neuromuscular junction is a chemical synapse essential for the
transmission of information from nervous system to skeletal muscle. There are many diseases related to neuromuscular junction
dysfunction, including myasthenia gravis, Lambert-Eaton myasthenic syndrome, congenital myasthenic syndromes, amyotrophic lateral
sclerosis, and spinal muscular atrophy. The pathophysiological mechanisms of these diseases have been investigated using many
animal models. Among them, mouse models are the most commonly used and have provided the majority of current data. Moreover,
advances in human induced pluripotent stem cell technology has resulted in new opportunities to study neuromuscular junction
disorders from both patients and healthy individuals. Currently, patient-specific induced pluripotent stem cells derived from motor
neurons have begun to be studied. These studies will help us achieve a more comprehensive understanding of diseases related to
neuromuscular junction disorders. We will describe the research models of neuromuscular junction disorders and provide an overview
of recent key findings.
Publisher
The Nencki Institute of Experimental Biology, Polish Academy of Sciences
Subject
General Medicine,General Neuroscience