Sp100 is a nuclear antigen localized in subnuclear domains called nuclear dots (NDs) or PML bodies. The Mus caroli variant shares structural and functional similarities with its human homolog, including interferon responsiveness and involvement in autoimmune responses . Recombinant production enables study of its biochemical interactions and diagnostic applications.
Gene Structure: The Mus caroli Sp100 gene spans 52 kb with 17 exons. Its promoter contains an interferon-stimulated response element (ISRE), critical for cytokine-induced expression .
Isoforms: Splice variants include HNPP-box and HMG1-containing forms, influencing nuclear localization and protein interactions .
Molecular Weight:
Protein Interactions: Binds HP1 family proteins (e.g., CBX5) via PxVxL motifs and Epstein-Barr virus EBNA-LP .
Autoantibody Binding: Recognized by IgG autoantibodies in PBC patients .
Interferon Response: IFN-α/β/γ upregulates Sp100 expression, enlarging nuclear dots .
Primary Biliary Cirrhosis: Anti-Sp100 antibodies present in 10–30% of PBC cases but lack disease specificity .
Limitations: Also found in SLE, hepatitis, and collagen diseases, necessitating clinical context for diagnosis .