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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2017
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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 |
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