Differential neuronal vulnerability to C9orf72 repeat expansion driven by Xbp1 transcription signature
Dunxin Shen,
Alec Vincent,
Evan Udine
et al.
Abstract:SummaryA G4C2 repeat expansion in the geneC9orf72(C9) is the most common genetic cause of sporadic and familial frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). What determines why cell death is triggered only in specific neuronal populations, while others remain ‘protected’ or are less susceptible to disease is still an open question. In particular, whether it is the transcriptional response to the accumulation of toxic insults or the initial cellular state that determines their vulnerab… Show more
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