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144,156,157 C9orf72 mutation, the leading genetic cause of ALS and FTD, disrupts microglial autophagy and triggers sustained activation of NLRP3 inflammasome and nuclear factor-B (NF-B) signaling in human-induced pluripotent stem cell-derived microglia-like cells (hiPSC-MG)

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The increase in free copper is also closely linked to neuroinflammation, as it promotes the activation of microglia and astrocytes, triggering an inflammatory response in the brain, which further exacerbates neuronal damage and cognitive decline (83)

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