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Gene Review

Sp100  -  nuclear antigen Sp100

Mus musculus

Synonyms: A430075G10Rik, Nuclear autoantigen Sp-100, Nuclear dot-associated Sp100 protein, Speckled 100 kDa
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Disease relevance of Sp100

  • In this study we present data which show that infection of HEp2 cells with influenza A virus, transformation of glial cells with SV40 DNA, and stimulation of PBL with mitogens affect the expression of the Sp100 autoantigen [1].
  • About 30% of patients suffering from the chronic autoimmune liver disease primary biliary cirrhosis produce autoantibodies against Sp100, a protein migrating in SDS-PAGE at a position corresponding to 100 kDa and located on discrete dot-shaped nuclear structures [1].

High impact information on Sp100


Biological context of Sp100

  • M. caroli represents the ancestral genomic organization of the cluster source components Sp100, Csprs and Ifi75: they are located close to each other in the same chromosome band (1D) [3].

Other interactions of Sp100

  • We identified and analyzed the genes Sp100, Csprs, and Ifi75 in two members of the genus Mus, M. musculus and M. caroli [5].
  • Thus, Sp100-rs arose relatively late in the evolution of the genus Mus, whereas Sp100 existed in the common ancestor of the human and the mouse [6].

Analytical, diagnostic and therapeutic context of Sp100

  • Replacement of the murine Sp100 gene by a defective copy is now feasible and should shed light on its function in an animal model [4].
  • As measured by an immunoblot-based ELISA the amount of Sp100 protein in INF-beta-treated cells (1000 IU/ml, 18 h) was eight to nine times higher than in untreated cells [1].


  1. IFN enhance expression of Sp100, an autoantigen in primary biliary cirrhosis. Guldner, H.H., Szostecki, C., Grötzinger, T., Will, H. J. Immunol. (1992) [Pubmed]
  2. A highly amplified mouse gene is homologous to the human interferon-responsive Sp100 gene encoding an autoantigen associated with nuclear dots. Grötzinger, T., Jensen, K., Guldner, H.H., Sternsdorf, T., Szostecki, C., Schwab, M., Savelyeva, L., Reich, B., Will, H. Mol. Cell. Biol. (1996) [Pubmed]
  3. Evolution of a 6-200 Mb long-range repeat cluster in the genus Mus. Traut, W., Rahn, I.M., Winking, H., Kunze, B., Weichehan, D. Chromosoma (2001) [Pubmed]
  4. Structure and expression of the murine Sp100 nuclear dot gene. Weichenhan, D., Kunze, B., Zacker, S., Traut, W., Winking, H. Genomics (1997) [Pubmed]
  5. Source and component genes of a 6-200 Mb gene cluster in the house mouse. Weichenhan, D., Kunze, B., Winking, H., van Geel, M., Osoegawa, K., de Jong, P.J., Traut, W. Mamm. Genome (2001) [Pubmed]
  6. Evolution by fusion and amplification: the murine Sp100-rs gene cluster. Weichenhan, D., Kunze, B., Traut, W., Winking, H. Cytogenet. Cell Genet. (1998) [Pubmed]
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