C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca(2+)-permeable AMPA receptor-mediated excitotoxicity

Mutations in C9ORF72 are the most common cause of familial amyotrophic lateral sclerosis (ALS). Here, through a combination of RNA-Seq and electrophysiological studies on induced pluripotent stem cell (iPSC)-derived motor neurons (MNs), we show that increased expression of GluA1 AMPA receptor (AMPAR...

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Detalles Bibliográficos
Autores principales: Selvaraj, Bhuvaneish T., Livesey, Matthew R., Zhao, Chen, Gregory, Jenna M., James, Owain T., Cleary, Elaine M., Chouhan, Amit K., Gane, Angus B., Perkins, Emma M., Dando, Owen, Lillico, Simon G., Lee, Youn-Bok, Nishimura, Agnes L., Poreci, Urjana, Thankamony, Sai, Pray, Meryll, Vasistha, Navneet A., Magnani, Dario, Borooah, Shyamanga, Burr, Karen, Story, David, McCampbell, Alexander, Shaw, Christopher E., Kind, Peter C., Aitman, Timothy J., Whitelaw, C. Bruce A., Wilmut, Ian, Smith, Colin, Miles, Gareth B., Hardingham, Giles E., Wyllie, David J. A., Chandran, Siddharthan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5783946/
https://www.ncbi.nlm.nih.gov/pubmed/29367641
http://dx.doi.org/10.1038/s41467-017-02729-0

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