TARDBP and amyotrophic lateral sclerosis: This splice variant, TDP-43 r.[106_196del], is upregulated approximately four-fold in ALS spinal cord compared to controls and is generated using non-canonical splice sites that skip the base pairs 106–196 in exon 2 of TARDBP, causing a frame shift and generating a premature termination codon 190 bp downstream from the first ATG.