Based on evidence from human studies, cell culture and animal models, the paradigm of pathological protein propagation in neurodegenerative diseases has been extended to include the concept that pathology arising from neurodegeneration-related proteins such as α-syn, amyloid-β, tau and TAR DNA-binding protein 43 (TDP43) may propagate in a prion-like fashion [9–13]. Here, TARDBP is linked to neurodegenerative disease.