The SP140L gene is thought to be a recent evolutionarily member of the SP100 family, formed through DNA rearrangements of the neighboring genes of SP100 and SP140.(22) A small subset of patients with primary biliary cirrhosis have been found to have autoantibodies against SP140L, which is not unexpected given our findings that the full-length protein is recognized by patients with various autoimmune diseases.(22) In contrast, the isolated PHD finger of SP140L demonstrated specific immunoreactivity in patients with anti-Mi2 autoantibodies. Here, SP140 is linked to autoimmune disease.