Autoantibodies to speckled protein family in primary biliary cholangitis

The autoantibody profile of primary biliary cholangitis (PBC) includes antinuclear antibodies (ANA) which are detectable by indirect immunofluorescence in more than 50% of PBC patients. One of the two immunofluorescence patterns which are historically considered PBC-specific is the so-called multiple nuclear dots (MND) targeting nuclear body proteins such as Sp100, Sp140, Sp140L proteins, promyelocytic leukemia protein (PML) and small ubiquitin-related modifier proteins (SUMO). It has been hypothesized a role of nuclear body protein alterations in immune disorders such as PBC, thus suggesting novel and more refined therapeutic approaches.

Automated summary

The autoantibody profile of primary biliary cholangitis (PBC) includes antinuclear antibodies (ANA) which are detectable by indirect immunofluorescence in over 50% of PBC patients. One of the historically considered PBC-specific immunofluorescence patterns is the multiple nuclear dots (MND), targeting nuclear body proteins and suggesting potential novel therapeutic approaches for immune disorders like PBC.