While it was reported [51] that TDP-43 protein bound the HERV-K LTR with an attendant increase in HERV-K(HML-2) transcription and RT activity, Manghera et al. [138] found wild-type TDP-43 bound the HERV-K(HML-2) promoter without activating its transcription, while overexpressed ALS-associated TDP-43 mutants promoted HERV-K(HML-2) protein aggregation and clearance from astrocytes (but not neurons) by stress granule formation and autophagy. Here, TARDBP is linked to amyotrophic lateral sclerosis.