Anti-endothelial cell antibodies from patients with limited cutaneous systemic sclerosis bind to centromeric protein B (CENP-B)

Clin Immunol. 2006 Aug;120(2):212-9. doi: 10.1016/j.clim.2006.02.006. Epub 2006 Mar 31.


By using a quantitative immunoblotting technique on protein extracts of human macrovascular and microvascular endothelial cells, we have analyzed the self-reactive repertoires of IgG from 20 patients with limited cutaneous SSc, 40 patients with diffuse SSc and 60 age- and sex-matched healthy controls. Serum IgG from 15/20 patients with limited cutaneous SSc and anti-centromere antibodies bound to at least one of the two 75- and 85-kDa protein bands in the different endothelial cell extracts, whereas IgG from healthy controls or patients with diffuse SSc did not. N-terminal sequencing of the 75- and 85-kDa bands identified CENP-B as the sole antigen in both bands. Moreover, IgG from all of the SSc patients who recognized the 75- and/or 85-kDa bands bound to a full-length recombinant CENP-B protein as assessed by ELISA, whereas IgG from other SSc patients did not. The main target of anti-endothelial cell antibodies in patients with limited cutaneous SSc is the nuclear and ubiquitous protein CENP-B.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibody Specificity
  • Antigens, Surface / chemistry
  • Antigens, Surface / immunology*
  • Autoantibodies / blood
  • Autoantibodies / chemistry
  • Autoantibodies / metabolism*
  • Cells, Cultured
  • Centromere Protein B / chemistry
  • Centromere Protein B / genetics
  • Centromere Protein B / immunology*
  • Endothelial Cells / chemistry
  • Endothelial Cells / immunology*
  • Female
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G / chemistry
  • Immunoglobulin G / metabolism
  • Male
  • Middle Aged
  • Recombinant Proteins / chemistry
  • Recombinant Proteins / genetics
  • Recombinant Proteins / immunology
  • Scleroderma, Diffuse / immunology*
  • Scleroderma, Limited / immunology*


  • Antigens, Surface
  • Autoantibodies
  • CENPB protein, human
  • Centromere Protein B
  • Immunoglobulin G
  • Recombinant Proteins