TDP-43, a nuclear RNA/DNA-binding protein capable of interacting with UG/TG repeat stretches of target RNAs/DNAs, plays a key role in regulation of transcription, alternative splicing, mRNA stability and transport, and microRNA biogenesis, actively involved in the pathogenesis of FTLD/ALS termed TDP-43 proteinopathy [31]. The gene discussed is TARDBP; the disease is proteostasis deficiencies.