C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity

Repeat expansion mutation in C9ORF72 is the most common cause of familial ALS. Here, the authors generate motor neurons from cells of patients with C9ORF72 mutations, and characterize changes in gene expression in these motor neurons compared to genetically corrected lines, which suggest that glutam...

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Auteurs principaux: Bhuvaneish T. Selvaraj, Matthew R. Livesey, Chen Zhao, Jenna M. Gregory, Owain T. James, Elaine M. Cleary, Amit K. Chouhan, Angus B. Gane, Emma M. Perkins, Owen Dando, Simon G. Lillico, Youn-Bok Lee, Agnes L. Nishimura, Urjana Poreci, Sai Thankamony, Meryll Pray, Navneet A. Vasistha, Dario Magnani, Shyamanga Borooah, Karen Burr, David Story, Alexander McCampbell, Christopher E. Shaw, Peter C. Kind, Timothy J. Aitman, C. Bruce A. Whitelaw, Ian Wilmut, Colin Smith, Gareth B. Miles, Giles E. Hardingham, David J. A. Wyllie, Siddharthan Chandran
Format: article
Langue:EN
Publié: Nature Portfolio 2018
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Accès en ligne:https://doaj.org/article/6a962a5b03a74471945829a86138ad2f
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