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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...

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Detalles Bibliográficos
Autores principales: Carlini, Maria J., Triplett, Marina K., Pellizzoni, Livio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2022
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