Numerous TDP-43-regulated CEs have been reproducibly detected in cellular and animal models, showing high specificity for TDP-LOF; furthermore, a number of these, including CEs in STMN2, UNC13A, HDGFL2 and AARS1, have been found specifically within neurons with TDP-43 pathology in post-mortem ALS and FTD samples (4–8, 16). This evidence concerns the gene TARDBP and frontotemporal dementia.