Reduced C9orf72 expression exacerbates polyGR toxicity in patient iPSC-derived motor neurons and a Type I protein arginine methyltransferase inhibitor reduces that toxicity

Reduced C9orf72 expression exacerbates polyGR toxicity in patient iPSC-derived motor neurons and a Type I protein arginine methyltransferase inhibitor reduces that toxicity

IntroductionIntronic repeat expansions in the C9orf72 gene are the most frequent known single genetic causes of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). These repeat expansions are believed to result in both loss-of-function and toxic gain-of-function. Gain-of-function...

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Bibliographic Details
Main Authors: Therese L. Dane, Anna L. Gill, Fernando G. Vieira, Kyle R. Denton
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-04-01
Series:Frontiers in Cellular Neuroscience
Subjects:
amyotrophic lateral sclerosis (ALS)
frontotemporal dementia (FTD)
C9orf72
protein arginine methyltransferase (PRMT)
asymmetric dimethylation
dipeptide repeat protein (DPR)
Online Access:https://www.frontiersin.org/articles/10.3389/fncel.2023.1134090/full

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https://www.frontiersin.org/articles/10.3389/fncel.2023.1134090/full

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