DOI: 10.1002/mus.10081. Possible pathogenic role of muscle cell dysfunction in motor neuron death in spinal muscular atrophy

Possible pathogenic role of muscle cell dysfunction in motor neuron death in spinal muscular atrophy

10.1002/mus.10081

Crossref journal-article

Wiley

Muscle & Nerve (311)

Abstract

AbstractWe have previously shown that myofibers formed by fusion of muscle satellite cells from spinal muscular atrophy (SMA) I or II undergo degeneration 1 to 3 weeks after innervation by rat embryonic spinal cord explants, whereas normal myofibers survive for several months. In the “muscle component” of the coculture, the only cells responsible for the degeneration are the SMA muscle satellite cells. Moreover, SMA muscle satellite cells do not fuse as rapidly as do normal muscle satellite cells. To determine whether death of muscle cells precedes that of motor neurons, we studied the origin and kinetics of release of apoptotic microparticles. In SMA cocultures, motor neuron apoptosis occurred before myofiber degeneration becomes visible, indicating that SMA myofibers were unable to sustain survival of motor neurons. In normal cocultures, motor neuron apoptosis occurred 4 days after innervation. However, it did not continue beyond 2 days. These results strengthen the hypothesis that SMA is due to a defect in neurotrophic muscle cell function. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25: 000–000, 2002

Bibliography

GuettierâSigrist, S., Hugel, B., Coupin, G., Freyssinet, J., Poindron, P., & Warter, J. (2002). Possible pathogenic role of muscle cell dysfunction in motor neuron death in spinal muscular atrophy. Muscle & Nerve, 25(5), 700â708. Portico.

Authors 6

Séverine Guettier‐Sigrist (first)
Bénédicte Hugel (additional)
Gilliane Coupin (additional)
Jean‐Marie Freyssinet (additional)
Philippe Poindron (additional)
Jean‐Marie Warter (additional)

References 36 Referenced 37

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Dates

Type	When
Created	23 years ago (Aug. 25, 2002, 7:24 p.m.)
Deposited	2 years ago (Aug. 30, 2023, 6:44 a.m.)
Indexed	3 months, 3 weeks ago (May 15, 2025, 6:48 a.m.)
Issued	23 years, 5 months ago (March 27, 2002)
Published	23 years, 5 months ago (March 27, 2002)
Published Online	23 years, 5 months ago (March 27, 2002)
Published Print	23 years, 4 months ago (May 1, 2002)

Funders 0

None

BibTeX

@article{Guettier_Sigrist_2002, title={Possible pathogenic role of muscle cell dysfunction in motor neuron death in spinal muscular atrophy}, volume={25}, ISSN={1097-4598}, url={http://dx.doi.org/10.1002/mus.10081}, DOI={10.1002/mus.10081}, number={5}, journal={Muscle & Nerve}, publisher={Wiley}, author={Guettier‐Sigrist, Séverine and Hugel, Bénédicte and Coupin, Gilliane and Freyssinet, Jean‐Marie and Poindron, Philippe and Warter, Jean‐Marie}, year={2002}, month=mar, pages={700–708} }

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