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DNA damage response defects induced by the formation of TDP-43 and mutant FUS cytoplasmic inclusions and their pharmacological rescue

28 May 2025

Abstract excerpt

Abstract Formation of cytoplasmic inclusions (CIs) of TDP-43 and FUS, along with DNA damage accumulation, is a hallmark of affected motor neurons in Amyotrophic Lateral Sclerosis (ALS). However, the impact of CIs on DNA damage response (DDR) and repair in this pathology remains unprobed. Here, we show that CIs of TDP-43 and FUS P525L , co-localizing with stress granules, lead to a dysfunctional DDR activation...

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PMID: 40437235
DOI: 10.1038/s41418-025-01530-7

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