Restoring calcium homeostasis in Purkinje cells arrests neurodegeneration and neuroinflammation in the ARSACS mouse model

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by mutations in SACS gene encoding sacsin, a huge protein highly expressed in cerebellar Purkinje cells (PCs). Patients with ARSACS, as well as mouse models, display early degeneration of PCs, but the underlying mechanisms...

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Detalles Bibliográficos
Autores principales: Del Bondio, Andrea, Longo, Fabiana, De Ritis, Daniele, Spirito, Erica, Podini, Paola, Brais, Bernard, Bachi, Angela, Quattrini, Angelo, Maltecca, Francesca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Clinical Investigation 2023
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10371240/
https://www.ncbi.nlm.nih.gov/pubmed/37159335
http://dx.doi.org/10.1172/jci.insight.163576

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