In fact, disruption of nucleolar phase transition in cell lines and animal models expressing dipeptide repeat proteins (DPR) produced by repeat-associated non-ATG (RAN) translation of an intronic hexanucleotide repeat expansion in the c9orf72 gene—the most common genetic cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) [20, 76]—causes DPR aggregation in the nucleoplasm, inhibits ribosome biogenesis, and results in cell death [28, 45, 52]. Here, C9orf72 is linked to amyotrophic lateral sclerosis.