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Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy

Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA is that not all motor neurons are equally affected...

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Detalles Bibliográficos
Autores principales: Boyd, Penelope J., Tu, Wen-Yo, Shorrock, Hannah K., Groen, Ewout J. N., Carter, Roderick N., Powis, Rachael A., Thomson, Sophie R., Thomson, Derek, Graham, Laura C., Motyl, Anna A. L., Wishart, Thomas M., Highley, J. Robin, Morton, Nicholas M., Becker, Thomas, Becker, Catherina G., Heath, Paul R., Gillingwater, Thomas H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5417717/
https://www.ncbi.nlm.nih.gov/pubmed/28426667
http://dx.doi.org/10.1371/journal.pgen.1006744