Rare mutations in the annexin A11 gene (ANXA11) may cause ALS with or without FTLD [53–57], and abnormal annexin A11 aggregates that occasionally co-localize with TDP-43 inclusions have been reported in brain/spinal cord autopsies from ALS cases with ANXA11 mutations [54, 57]. This evidence concerns the gene ANXA11 and amyotrophic lateral sclerosis.