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Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts

Dysregulation of RNA metabolism represents an important pathogenetic mechanism in both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) due to the involvement of the DNA/RNA-binding proteins TDP-43 and FUS and, more recently, of C9ORF72. A potential link between dysregulation of...

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Detalles Bibliográficos
Autores principales: Onesto, Elisa, Colombrita, Claudia, Gumina, Valentina, Borghi, Maria Orietta, Dusi, Sabrina, Doretti, Alberto, Fagiolari, Gigliola, Invernizzi, Federica, Moggio, Maurizio, Tiranti, Valeria, Silani, Vincenzo, Ratti, Antonia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4858818/
https://www.ncbi.nlm.nih.gov/pubmed/27151080
http://dx.doi.org/10.1186/s40478-016-0316-5