This genetic insult is thought to cause ALS/FTD via three non-mutually exclusive mechanisms: (1) loss of function due to decreased expression of C9ORF72 protein, (2) RNA toxicity from bidirectionally transcribed sense (GGGGCC) and antisense (CCCCGG) transcripts, and (3) proteotoxicity from dipeptide repeat (DPR) proteins produced from the expanded nucleotide repeats3. This evidence concerns the gene C9orf72 and frontotemporal dementia.