Cargando…
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...
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 |
Ejemplares similares
-
Reprogramming fibroblasts and peripheral blood cells from a C9ORF72 patient: A proof‐of‐principle study
por: Bardelli, Donatella, et al.
Publicado: (2020) -
Inter-Species Differences in Regulation of the Progranulin–Sortilin Axis in TDP-43 Cell Models of Neurodegeneration
por: Gumina, Valentina, et al.
Publicado: (2019) -
Motor, cognitive and behavioural profiles of C9orf72 expansion-related amyotrophic lateral sclerosis
por: Colombo, Eleonora, et al.
Publicado: (2022) -
Correction to: Motor, cognitive and behavioural profiles of C9orf72 expansion-related amyotrophic lateral sclerosis
por: Colombo, Eleonora, et al.
Publicado: (2023) -
Pantethine treatment is effective in recovering the disease phenotype induced by ketogenic diet in a pantothenate kinase-associated neurodegeneration mouse model
por: Brunetti, Dario, et al.
Publicado: (2014)