Although the function of C9orf72 remains unknown, C9orf72 knockout rodent studies demonstrate a systemic proinflammatory state, severe autoimmune disease [50,51,52,83,84], mild neuroinflammation characterised by increased expression of IL6 and IL1β in microglia, and an upregulation of inflammatory genes in the spinal cord compared to control mice [85]. This evidence concerns the gene C9orf72 and autoimmune disease.