In a model of amyotrophic lateral sclerosis (ALS) caused by C9orf72 gene mutations, the pathogenic poly-dipeptide GA50 directly binds to and inhibits SQOR, leading to excessive NLRP3 inflammasome activation in microglia and consequent mitochondrial dysfunction (Fu et al., 2023). The gene discussed is C9orf72; the disease is amyotrophic lateral sclerosis.