In C9ORF72-associated ALS/frontotemporal dementia models, dipeptide repeat expression triggered marked increases in ECM proteins such as collagen VI (COL6A1); overexpression of TGF-β1 or COL6A1 enhanced neuronal resistance to excitotoxicity, whereas their suppression exacerbated degeneration [40]. Here, COL6A1 is linked to frontotemporal dementia.