The present study shows that UPF1 expression regulates the DPR- or HRE-induced neurodegenerative phenotypes of C9orf72-ALS transgenic D. melanogaster models, including eye symptoms (Xu et al., 2019; Ortega et al., 2020; Sun et al., 2020). The gene discussed is UPF1; the disease is amyotrophic lateral sclerosis.