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Neuromuscular denervation and deafferentation but not motor neuron death are disease features in the Smn(2B/)(-) mouse model of SMA
Spinal muscular atrophy (SMA) is a neurodegenerative disease characterized by loss of motor neurons and skeletal muscle atrophy which is caused by ubiquitous deficiency in the survival motor neuron (SMN) protein. Several cellular defects contribute to sensory-motor circuit pathology in SMA mice, but...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9342749/ https://www.ncbi.nlm.nih.gov/pubmed/35913953 http://dx.doi.org/10.1371/journal.pone.0267990 |