Impairment of Mitochondrial Calcium Buffering Links Mutations in C9ORF72 and TARDBP in iPS-Derived Motor Neurons from Patients with ALS/FTD

TDP-43 dysfunction is common to 97% of amyotrophic lateral sclerosis (ALS) cases, including those with mutations in C9orf72. To investigate how C9ORF72 mutations drive cellular pathology in ALS and to identify convergent mechanisms between C9ORF72 and TARDBP mutations, we analyzed motor neurons (MNs...

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Detalles Bibliográficos
Autores principales: Dafinca, Ruxandra, Barbagallo, Paola, Farrimond, Lucy, Candalija, Ana, Scaber, Jakub, Ababneh, Nida'a A., Sathyaprakash, Chaitra, Vowles, Jane, Cowley, Sally A., Talbot, Kevin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7220989/
https://www.ncbi.nlm.nih.gov/pubmed/32330447
http://dx.doi.org/10.1016/j.stemcr.2020.03.023

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