Anti-sp100 antibodies are found in association with primary biliary cirrhosis. The autoimmune target of anti-sp100 is the sp100 nuclear antigen which was identified by its association with primary biliary cirrhosis. 20-30% of patients suffering from primary biliary cirrhosis have sp100 Abs. The sera of these patients exhibit a characteristic "nuclear dots" pattern in indirect immunofluorescence (IIF) on Hep-2 cells.
Immunodominant regions of sp100
- Antigenic region 1 - positions 296-311 - Sequence (I K K E K P F S N S K V E C Q A)
- Antigenic region 2 - positions 332-351 - Sequence (E G S T D V D E P L E V F I S A P R S E)
(Bolded areas represent the core epitope.)
- Szostecki C, Guldner HH, Netter HJ, Will H (1990). "Isolation and characterization of cDNA encoding a human nuclear antigen predominantly recognized by autoantibodies from patients with primary biliary cirrhosis". J. Immunol. 145 (12): 4338–47. PMID 2258622.
- Blüthner M, Schäfer C, Schneider C, Bautz FA (1999). "Identification of major linear epitopes on the sp100 nuclear PBC autoantigen by the gene-fragment phage-display technology". Autoimmunity 29 (1): 33–42. doi:10.3109/08916939908995970. PMID 10052683.