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Inhibition of autophagy delays motoneuron degeneration and extends lifespan in a mouse model of spinal muscular atrophy

Spinal muscular atrophy (SMA) is a recessive autosomal neuromuscular disease, due to homozygous mutations or deletions in the telomeric survival motoneuron gene 1 (SMN1). SMA is characterized by motor impairment, muscle atrophy, and premature death following motor neuron (MN) degeneration. Emerging...

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Detalles Bibliográficos
Autores principales: Piras, Antonio, Schiaffino, Lorenzo, Boido, Marina, Valsecchi, Valeria, Guglielmotto, Michela, De Amicis, Elena, Puyal, Julien, Garcera, Ana, Tamagno, Elena, Soler, Rosa M, Vercelli, Alessandro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5870600/
https://www.ncbi.nlm.nih.gov/pubmed/29259166
http://dx.doi.org/10.1038/s41419-017-0086-4