Canonical cGAS/STING activation was observed in ALS/FTD patient iPSC-derived neurons, layer V cortical motor neurons in C9orf72 repeat expansion transgenic mice, as well as cortical and spinal motor neurons in postmortem ALS tissue, demonstrating that neuronal STING activation, resulting in Tnfα, Il6, and Cxcl10 production, drives pathogenesis in ALS/FTD models (127). Here, C9orf72 is linked to frontotemporal dementia.