Numerous Hem1-deficient mouse models demonstrated systemic autoimmunity and inflammation parallel to that seen in patients, including splenomegaly, immune complex lupus glomerulonephritis, elevated proinflammatory markers (IL-6, IFNγ, and MRP8/14), and autoreactive plasmablasts with elevated serum dsDNA autoantibodies [9, 41]. This evidence concerns the gene NCKAP1L and Autoimmunity.