Article
The Ku80-p53-SIRT1 axis in DNA damage response contributes to sporadic and familial ALS and FTD.
Nature communications - 20 Dec 2025
Jun Yong-Woo, Lee Soojin, Almeida Sandra, Freude Kristine K, Ichida Justin K, Gao Fen-Biao
Abstract excerpt
Although TDP-43 pathology is found in most sporadic and familial ALS and FTD cases, other shared pathogenic mechanisms remain largely unknown. Here we show that SIRT1 levels are decreased and acetylated p53 levels are increased in iPSC-derived neurons from sALS patients and with the FTD3-causing CHMP2B mutation. Ectopic expression of SIRT1 in these patient neurons rescues neurodegeneration and reduces acetylated...
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