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Small-molecule flunarizine increases SMN protein in nuclear Cajal bodies and motor function in a mouse model of spinal muscular atrophy
The hereditary neurodegenerative disorder spinal muscular atrophy (SMA) is characterized by the loss of spinal cord motor neurons and skeletal muscle atrophy. SMA is caused by mutations of the survival motor neuron (SMN) gene leading to a decrease in SMN protein levels. The SMN deficiency alters nuc...
Autores principales: | Sapaly, Delphine, Dos Santos, Matthieu, Delers, Perrine, Biondi, Olivier, Quérol, Gwendoline, Houdebine, Léo, Khoobarry, Kevinee, Girardet, François, Burlet, Philippe, Armand, Anne-Sophie, Chanoine, Christophe, Bureau, Jean-François, Charbonnier, Frédéric, Lefebvre, Suzie |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5794986/ https://www.ncbi.nlm.nih.gov/pubmed/29391529 http://dx.doi.org/10.1038/s41598-018-20219-1 |
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