Nusinersen ameliorates motor function and prevents motoneuron Cajal body disassembly and abnormal poly(A) RNA distribution in a SMA mouse model

Spinal muscular atrophy (SMA) is a devastating autosomal recessive neuromuscular disease characterized by degeneration of spinal cord alpha motor neurons (αMNs). SMA is caused by the homozygous deletion or mutation of the survival motor neuron 1 (SMN1) gene, resulting in reduced expression of SMN pr...

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Detalles Bibliográficos
Autores principales: Berciano, María T., Puente-Bedia, Alba, Medina-Samamé, Almudena, Rodríguez-Rey, José C., Calderó, Jordi, Lafarga, Miguel, Tapia, Olga
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7330045/
https://www.ncbi.nlm.nih.gov/pubmed/32612161
http://dx.doi.org/10.1038/s41598-020-67569-3

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