Clinical relevance of terminal Schwann cells: An overlooked component of the neuromuscular junction

The terminal Schwann cell (tSC), a type of nonmyelinating Schwann cell, is a significant yet relatively understudied component of the neuromuscular junction. In addition to reviewing the role tSCs play on formation, maintenance, and remodeling of the synapse, we review studies that implicate tSCs in...

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Published inJournal of neuroscience research Vol. 96; no. 7; pp. 1125 - 1135
Main Authors Santosa, Katherine B., Keane, Alexandra M., Jablonka‐Shariff, Albina, Vannucci, Bianca, Snyder‐Warwick, Alison K.
Format Journal Article
LanguageEnglish
Published United States Wiley Subscription Services, Inc 01.07.2018
Subjects
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - pathology
Animals
Atrophy
Degeneration
glia
Humans
Medical innovations
Miller Fisher Syndrome - pathology
Miller-Fisher syndrome
Muscular Atrophy, Spinal - pathology
Neuromuscular diseases
Neuromuscular Diseases - pathology
neuromuscular junction
Neuromuscular Junction - physiology
Pathogenesis
Regeneration
Schwann cells
Schwann Cells - metabolism
Schwann Cells - pathology
Schwann Cells - physiology
Spinal muscular atrophy
neuromuscular junction
glia
Schwann cells
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Summary:The terminal Schwann cell (tSC), a type of nonmyelinating Schwann cell, is a significant yet relatively understudied component of the neuromuscular junction. In addition to reviewing the role tSCs play on formation, maintenance, and remodeling of the synapse, we review studies that implicate tSCs in neuromuscular diseases including spinal muscular atrophy, Miller–Fisher syndrome, and amyotrophic lateral sclerosis, among others. We also discuss the importance of these cells on degeneration and regeneration after nerve injury. Knowledge of tSC biology may improve our understanding of disease pathogenesis and help us identify new and innovative therapeutic strategies for the many patients who suffer from neuromuscular disorders and nerve injuries.
Bibliography:Supported by the Plastic Surgery Foundation and the NIH National Institute of Neurological Disorders and Stroke Awards: F32NS098561 (to K.B.S.) and K08NS096232 (to A.K.S.W.)
Funding Information
Terminal Schwann cells (tSCs) are nonmyelinating Schwann cells that reside at the neuromuscular junction and are important for synaptic development, maintenance, and remodeling. In addition to their roles in degeneration and regeneration after nerve injury, tSCs have been implicated in neuromuscular diseases, including spinal muscular atrophy, Miller–Fisher syndrome, and amyotrophic lateral sclerosis. An improved understanding of tSCs may lead to novel therapeutic strategies for these neuromuscular diseases and for nerve injuries.
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ISSN:0360-4012
1097-4547
DOI:10.1002/jnr.24231