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Table 1 Relationship between nuclear pore complex proteins, antinuclear antibodies giving the “multiple nuclear dots” immunofluorescence pattern, and clinical significance in patients with primary biliary cholangitis

From: Autoantibodies to speckled protein family in primary biliary cholangitis

Nuclear structures Indirect immunofluorescence pattern Autoantigen targets Autoantibodies in PBC
Prevalence (%) Specificity Worse prognosis
Nuclear Bodies MNDa Sp100 17–41 High To be confirmed
PML 19 High No
Sp140 15 High No
Sp140L 1.5 Not assessed Not assessed
SUMO-1 15 Not assessed Not assessed
SUMO-2 42 Not assessed Not assessed
  1. Sp100 Sp100 nuclear body component, PML promyelocytic leukemia protein, Sp140 Sp140 nuclear body component, Sp140L Sp140L protein, MND multiple nuclear dots pattern, SUMO small ubiquitin-related modifiers
  2. aMND pattern detected by indirect immunofluorescence using fluorescein-conjugate anti-human total Ig and anti-human IgG subclasses (IgG1, IgG2, IgG3, IgG4) as secondary antibody was found in 16% and 31%, respectively [1, 2]