Spontaneous occurrence of anti-fibrillin-1 autoantibodies in tight-skin mice

Chihiro Murai, Shinichiro Saito, Kuppuswamy N. Kasturi, Constantin A. Bona

Research output: Contribution to journalArticlepeer-review

50 Citations (Scopus)


Tight-skin (TSK) mouse represents an experimental for systemic sclerosis, displaying cutaneous hyperplasia, connective tissue alterations in the internal organs and developing autoantibodies against several scleroderma target autoantigens. TSK mouse syndrome is associated with a mutation in fibrillin-1 (Fbn-1), the major component of 10 nm microfibrils. Here, we have investigated whether TSK mouse develops autoimmunity to Fbn-1 similar to scleroderma target autoantigens. Our results show that anti-Fbn-1 IgG autoantibodies are present in high titer in many TSK mice. Specificity of these antibodies was confirmed by competitive inhibition assays and Western blotting analysis using recombinant human Fbn-1 protein. TSK mouse autoantibodies recognize a conserved epitope present in the C region of Fbn-1. These results indicate the presence of Fbn-1 specific T and B cells in TSK mouse repertoire.

Original languageEnglish
Pages (from-to)151-155
Number of pages5
Issue number3
Publication statusPublished - 1998
Externally publishedYes


  • Autoantibodies
  • Fibrillin-1
  • Tight-skin mouse

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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