The knockdown of EXOSC10 in fibroblasts derived from patients with C9orf72 FTLD/ALS resulted in intracellular repeat RNA accumulation and increased nuclear RNA foci, suggesting that the EXOSC10/RNA exosome complex is involved in the metabolism of endogenous repeat RNA in patient-derived cells. The gene discussed is EXOSC10; the disease is amyotrophic lateral sclerosis.