Amongst the RBPs linked to neurodegeneration, TDP-43 has received the most attention, as pathogenic accumulations of the protein are found in the majority of ALS patients, as well as tau-negative FTD and a proportion of AD [2,3], and have been shown to promote neurodegeneration in cell and animal models [4,5]. The gene discussed is TARDBP; the disease is frontotemporal dementia.