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Improvement of Neuromuscular Synaptic Phenotypes without Enhanced Survival and Motor Function in Severe Spinal Muscular Atrophy Mice Selectively Rescued in Motor Neurons
In the inherited childhood neuromuscular disease spinal muscular atrophy (SMA), lower motor neuron death and severe muscle weakness result from the reduction of the ubiquitously expressed protein survival of motor neuron (SMN). Although SMA mice recapitulate many features of the human disease, it ha...
Autores principales: | Paez-Colasante, Ximena, Seaberg, Bonnie, Martinez, Tara L., Kong, Lingling, Sumner, Charlotte J., Rimer, Mendell |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3781079/ https://www.ncbi.nlm.nih.gov/pubmed/24086650 http://dx.doi.org/10.1371/journal.pone.0075866 |
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