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In vitro Modelling of Human Healthy and ALS Proprioceptive Sensory Neurons in the Neuromuscular System

45 Pages Posted: 12 Jan 2022 Publication Status: Review Complete

See all articles by Maider Badiola-Mateos

Maider Badiola-Mateos

Barcelona Institute of Science and Technology (BIST) - Institute for Bioengineering of Catalonia (IBEC)

Tatsuya Osaki

Massachusetts Institute of Technology (MIT) - Department of Biological Engineering

Roger D. Kamm

Massachusetts Institute of Technology (MIT) - Department of Mechanical Engineering; Massachusetts Institute of Technology (MIT) - Department of Biological Engineering

Josep Samitier

Barcelona Institute of Science and Technology (BIST) - Institute for Bioengineering of Catalonia (IBEC)

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Abstract

Proprioceptive sensory neurons (pSN) are an essential and undervalued part of the neuromuscular circuit. A developed 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 coculture platforms. The obtained pSN spheroids cultured with human skeletal myocytes recreated afferent neuromuscular connections, in which we observed the formation of annulospiral wrappinglike structures between TrkC+ neurons and a multinucleated muscle fiber, presenting synaptic bouton-like structures in the contact point. The genetic profile comparative analysis performed in healthy and ALS hNSC differentiated to pSN showed that basal levels of ETV1, critical for motor feedback from pSN, were much lower for ALS samples and that the pSN genetic profile varies between healthy and ALS samples, suggesting a strong involvement of pSN in ALS pathology development and progression.

Keywords: neuromuscular systems, sensory neurons, ALS, In vitro 3D neuromuscular models

Suggested Citation

Badiola-Mateos, Maider and Osaki, Tatsuya and Kamm, Roger D. and Samitier, Josep, In vitro Modelling of Human Healthy and ALS Proprioceptive Sensory Neurons in the Neuromuscular System. Available at SSRN: https://ssrn.com/abstract=4007585 or http://dx.doi.org/10.2139/ssrn.4007585
This version of the paper has not been formally peer reviewed.

Maider Badiola-Mateos

Barcelona Institute of Science and Technology (BIST) - Institute for Bioengineering of Catalonia (IBEC) ( email )

Barcelona
Spain

Tatsuya Osaki

Massachusetts Institute of Technology (MIT) - Department of Biological Engineering ( email )

77 Massachusetts Avenue
50 Memorial Drive
Cambridge, MA 02139-4307
United States

Roger D. Kamm

Massachusetts Institute of Technology (MIT) - Department of Mechanical Engineering ( email )

United States

Massachusetts Institute of Technology (MIT) - Department of Biological Engineering ( email )

77 Massachusetts Avenue
50 Memorial Drive
Cambridge, MA 02139-4307
United States

Josep Samitier (Contact Author)

Barcelona Institute of Science and Technology (BIST) - Institute for Bioengineering of Catalonia (IBEC) ( email )

Barcelona
Spain

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