Structure and expression of the murine Sp100 nuclear dot gene.

The human SP100 gene encodes an autoantigen that colocalizes with two other proteins, PML and NDP52, in distinct nuclear domains, called "nuclear dots" (NDs). NDs do not overlap with other known subnuclear structures, and their function is still unknown. Patients suffering from the autoimmune disease primary biliary cirrhosis often produce antibodies against the SP100 protein. The present study describes the structure and expression of the murine Sp100 gene. In the species Mus caroli, Sp100 consists of 17 exons that are distributed over a range of 52 kb. The human and murine Sp100 promoters are very similar, and both harbor an interferon-stimulated response element. Like its human counterpart, the murine Sp100 gene is responsive to interferon treatment. The house mouse, Mus musculus, harbors the Sp100 gene and a second gene with homology to Sp100, the multicopy Sp100-rs gene. However, in contrast to the genuine mouse homolog, Sp100-rs shares only segmental homology with the human Sp100 gene. Replacement of the murine Sp100 gene by a defective copy is now feasible and should shed light on its function in an animal model.