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Antinuclear antibodies as ancillary markers in primary biliary cirrhosis.

Abstract
Antimitochondrial antibodies are the serological hallmark of primary biliary cirrhosis (PBC). Besides antimitochondrial antibodies, the autoantibody profile of PBC includes antinuclear antibodies (ANA) which are detectable by indirect immunofluorescence in up to 50% of PBC patients. Two immunofluorescence patterns are considered 'PBC-specific': the multiple nuclear dots and rim-like/membranous patterns. The target antigens of the multiple nuclear dots pattern have been identified as Sp100 and promyelocytic leukemia protein, whereas the rim-like/membranous pattern is given by autoantibodies recognizing multiple proteins such as gp210, nucleoporin p62 and the lamin B receptor. Other ANA, especially those already known in the rheumatological setting, such as anticentromere, anti-SSA/Ro and anti-dsDNA antibodies, can be frequently found in PBC, often coexisting in the same patient. In this article, we will report on recent progress in the antigenic characterization of ANA in PBC, their detection with both traditional assays and Western blot/ELISA with molecularly defined nuclear antigens, and we will discuss their clinical significance.
AuthorsAlessandro Granito, Paolo Muratori, Chiara Quarneti, Georgios Pappas, Ronny Cicola, Luigi Muratori
JournalExpert review of molecular diagnostics (Expert Rev Mol Diagn) Vol. 12 Issue 1 Pg. 65-74 (Jan 2012) ISSN: 1744-8352 [Electronic] England
PMID22133120 (Publication Type: Journal Article, Review)
Chemical References
  • Antibodies, Antinuclear
  • Antigens, Nuclear
  • Autoantigens
  • Biomarkers
  • Membrane Glycoproteins
  • NUP210 protein, human
  • Nuclear Pore Complex Proteins
  • Nuclear Proteins
  • Receptors, Cytoplasmic and Nuclear
  • Transcription Factors
  • Tumor Suppressor Proteins
  • lamin B receptor
  • nuclear pore protein p62
  • Sp100 protein, human
  • PML protein, human
Topics
  • Antibodies, Antinuclear (immunology)
  • Antigens, Nuclear (immunology)
  • Autoantigens (immunology)
  • Biomarkers (metabolism)
  • Blotting, Western (methods)
  • Enzyme-Linked Immunosorbent Assay (methods)
  • Epitope Mapping
  • Fluorescent Antibody Technique, Indirect (methods)
  • Humans
  • Liver Cirrhosis, Biliary (diagnosis, immunology)
  • Membrane Glycoproteins (immunology)
  • Nuclear Pore Complex Proteins (immunology)
  • Nuclear Proteins (immunology)
  • Prognosis
  • Receptors, Cytoplasmic and Nuclear (immunology)
  • Transcription Factors (immunology)
  • Tumor Suppressor Proteins (immunology)

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