In support of this, transposable elements, which are broadly targeted and silenced by the multifunctional RNA binding protein TDP-43 are well-known to be de-repressed in TDP-43 pathology-associated neurodegeneration, such as in the spectrum of age-related fronto-temporal lobar dementia (FTLD) and amyotrophic lateral sclerosis (ALS) [108]. Here, TARDBP is linked to amyotrophic lateral sclerosis.