SFPQ and amyotrophic lateral sclerosis: Indeed, SFPQ-knockdown mice exhibit FTLD-like phenotypes35 and motor neurons lacking SFPQ degenerate by exhibiting a ‘dying back’ phenotype reminiscent of ALS in zebrafish disease models.41 However, it is important to also consider intranuclear spatial dissociation of RBP complexes in the molecular pathogenesis of FTLD spectrum disorders.