Calpain Inhibition Increases SMN Protein in Spinal Cord Motoneurons and Ameliorates the Spinal Muscular Atrophy Phenotype in Mice

Spinal muscular atrophy (SMA), a leading genetic cause of infant death, is caused by the loss of survival motor neuron 1 (SMN1) gene. SMA is characterized by the degeneration and loss of spinal cord motoneurons (MNs), muscular atrophy, and weakness. SMN2 is the centromeric duplication of the SMN gen...

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Detalles Bibliográficos
Autores principales: de la Fuente, Sandra, Sansa, Alba, Periyakaruppiah, Ambika, Garcera, Ana, Soler, Rosa M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6505520/
https://www.ncbi.nlm.nih.gov/pubmed/30327977
http://dx.doi.org/10.1007/s12035-018-1379-z

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6505520/
https://www.ncbi.nlm.nih.gov/pubmed/30327977
http://dx.doi.org/10.1007/s12035-018-1379-z

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