DNA2 and amyotrophic lateral sclerosis: As expected, given the difference in baseline mtCN, this led to a significant difference between patient and control derived cells: after treatment with 75 μm of C5 DNA2 inhibitor the number of nuclei was reduced for control iNeurons (FC = 0.33, p = 0.006, Welch's t-test, Fig. 4B) but not for C9ORF72-ALS iNeurons (FC = 0.89, p = 0.41, Fig. 4B); similarly MMP was reduced for control iNeurons (FC = 0.58, p = 0.003, Welch's t-test, Fig. 4C) but not for C9ORF72-ALS iNeurons (FC = 0.93, p = 0.28, Fig. 4C).