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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...
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 |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2017
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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 |
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