Ontology highlight
ABSTRACT:
SUBMITTER: Zhao C
PROVIDER: S-EPMC7078830 | biostudies-literature | 2020 May
REPOSITORIES: biostudies-literature
Zhao Chen C Devlin Anna-Claire AC Chouhan Amit K AK Selvaraj Bhuvaneish T BT Stavrou Maria M Burr Karen K Brivio Veronica V He Xin X Mehta Arpan R AR Story David D Shaw Christopher E CE Dando Owen O Hardingham Giles E GE Miles Gareth B GB Chandran Siddharthan S
Glia 20191216 5
Mutations in C9orf72 are the most common genetic cause of amyotrophic lateral sclerosis (ALS). Accumulating evidence implicates astrocytes as important non-cell autonomous contributors to ALS pathogenesis, although the potential deleterious effects of astrocytes on the function of motor neurons remains to be determined in a completely humanized model of C9orf72-mediated ALS. Here, we use a human iPSC-based model to study the cell autonomous and non-autonomous consequences of mutant C9orf72 expre ...[more]