Expression of a cryptic exon upstream of the TDP-43 binding site, ELAVL3 CE 4a, is seen when TDP-43 is reduced in a TDP-43 knock-down cellular model and in TDP-43 negative neuronal nuclei from frontotemporal lobar degeneration frontal cortex (Fig. 1b) [3, 10]. This evidence concerns the gene TARDBP and frontotemporal dementia.