Sp100

Cat. No.Available inproduct_image_58
189000.1 mg
189011.0 mg
 


Diseases:
Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is an autoimmune chronic cholestatic liver disease characterized by destruction of intrahepatic bile ducts and development of cirrhosis and liver failure. Serologically, PBC is characterized by the presence of antimitochondrial (AMA, M2) antibodies. The immunological profil of PBC also includes antinuclear antibodies (ANA), presenting two distinct patterns, multiple nuclear dots (MND) and rim-like/membranous patterns in IIF. Sp100 and promyelocytic leukemia antigen account for the MND pattern, and gp210, nucleoporin p62 and lamin B receptor are associated with nuclear membrane staining.

Sp100 is the main antigenic target of MND. This 54-kDa nuclear protein has transcription-enhancing activity and is upregulated by interferon. Although the high specificity of anti-gp210 for PBC is generally accepted, the true diagnostic role of Sp100 is still under debate since also a number of SLE sera and those of patients with other hepatic or non-hepatic disorders seem to be seropositive for Sp100 antibodies. In any case, anti-Sp100 is more frequent in liver patients and has been significantly more often detected in PBC, for which it can be regarded as a highly specific serological marker.

Sp100 antigen from DIARECT is produced in the baculovirus / insect cell expression system.