Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

GGGGCC repeat expansions in C9ORF72 are the most common genetic cause of both ALS and FTD. To uncover underlying pathogenic mechanisms, we found DNA damage was greater, in an age-dependent manner, in motor neurons differentiated from iPSCs of multiple C9ORF72 patients than control neurons. Ectopic e...

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Bibliografiska uppgifter
I publikationen:Neuron
Huvudupphovsmän: Lopez-Gonzalez, Rodrigo, Lu, Yubing, Gendron, Tania F., Karydas, Anna, Tran, Helene, Yang, Dejun, Petrucelli, Leonard, Miller, Bruce L., Almeida, Sandra, Gao, Fen-Biao
Materialtyp: Artigo
Språk:Inglês
Publicerad: 2016
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Article
Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC5111366/
https://ncbi.nlm.nih.gov/pubmed/27720481
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.neuron.2016.09.015
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