To explore the relationship between TDP-43 and annexin A11 in neurodegenerative diseases, we first performed a genetic analysis to identify rare ANXA11 variants in a cohort of 822 CNDR autopsies, followed by immunohistochemistry to assess for the presence of annexin A11 aggregate pathology in the cases carrying the rare ANXA11 variants. This evidence concerns the gene TARDBP and neurodegenerative disease.