Anti-sp100 antibodies
Encyclopedia
Primary biliary cirrhosis
Primary biliary cirrhosis, often abbreviated PBC, is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts within the liver. When these ducts are damaged, bile builds up in the liver and over time damages the tissue. This can lead to scarring,...
. The autoimmune target of anti-sp100 is the sp100 nuclear antigen
Sp100 nuclear antigen
Sp100 nuclear antigen is an interferon stimulated antigen found in the bile duct of primary biliary cirrhosis. Histologically sp100 'dots' regions of the cell nucleus. Viral infection and mitogens affect the expression of the Sp100 autoantigen...
which was identified by its association with primary biliary cirrhosis
Primary biliary cirrhosis
Primary biliary cirrhosis, often abbreviated PBC, is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts within the liver. When these ducts are damaged, bile builds up in the liver and over time damages the tissue. This can lead to scarring,...
. 20-30% of patients suffering from primary biliary cirrhosis have sp100 Abs.
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)