TARDBP and amyotrophic lateral sclerosis: A pathological hallmark of ALS is the abnormal aggregation into inclusions of TAR DNA-binding protein 43 (TDP-43), a DNA/RNA-binding protein localized primarily in the nucleus, accompanied by various post-translational modifications of TDP-43 such as ubiquitination, phosphorylation, and truncation in the cytoplasm of affected neurons.