RBM45 accumulates in inclusion bodies in ALS and FTLD patients. RBM45 co-localizes with TDP-43's cytoplasmic aggregates. No RBM45 mutations in ALS have been reported yet. Mutations in RBM45 show propensity to form cytoplasmic aggregates which recruit TDP-43, and impair mitochondrial functions. Here, RBM45 is linked to amyotrophic lateral sclerosis.