Author:
Badiola-Mateos Maider,Osaki Tatsuya,Kamm Roger Dale,Samitier Josep
Abstract
AbstractProprioceptive sensory neurons (pSN) are an essential and undervalued part of the neuromuscular circuit. A protocol to differentiate healthy and amyotrophic lateral sclerosis (ALS) human neural stem cells (hNSC) into pSN, and their comparison with the motor neuron (MN) differentiation process from the same hNSC sources, facilitated the development of in vitro co-culture platforms. The obtained pSN spheroids cultured interact with human skeletal myocytes showing the formation of annulospiral wrapping-like structures between TrkC + neurons and a multinucleated muscle fibre, presenting synaptic bouton-like structures in the contact point. The comparative analysis of the genetic profile performed in healthy and sporadic ALS hNSC differentiated to pSN suggested that basal levels of ETV1, critical for motor feedback from pSN, were much lower for ALS samples and that the differences between healthy and ALS samples, suggest the involvement of pSN in ALS pathology development and progression.
Funder
Generalitat de Catalunya
Ministerio de Ciencia e Innovación
Japan Society for the Promotion of Science
National Science Foundation
Publisher
Springer Science and Business Media LLC
Reference99 articles.
1. Badiola-Mateos, M., Hervera, A., del Río, J. A. & Samitier, J. Challenges and future prospects on 3D in-vitro modeling of the neuromuscular circuit. Front. Bioeng. Biotechnol. 6, 194 (2018).
2. Hunter, G. et al. Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy. Hum. Mol. Genet. 25, 2853–2861 (2016).
3. Vilmont, V., Cadot, B., Ouanounou, G. & Gomes, E. R. A system for studying mechanisms of neuromuscular junction development and maintenance. Development 143, 2464–2477 (2016).
4. Santosa, K. B., Keane, A. M., Jablonka-Shariff, A., Vannucci, B. & Snyder-Warwick, A. K. Clinical relevance of terminal Schwann cells: An overlooked component of the neuromuscular junction. J. Neurosci. Res. 96, 1125–1135 (2018).
5. Trias, E. et al. Schwann cells orchestrate peripheral nerve inflammation through the expression of CSF1, IL-34, and SCF in amyotrophic lateral sclerosis. Glia 68, 1165–1181 (2020).
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