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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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
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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