TARDBP and frontotemporal dementia: In contrast, transactive response DNA-binding protein of 43 kDa (TDP-43), which is a candidate gene responsible for diverse neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), preferentially binds RNAs via a GU dinucleotide repeat (GU)n element and regulates mRNA splicing, stability, and translational repression.