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 Jan 1


    • Autoantibodies
    • Fibrillin-1
    • Tight-skin mouse

    ASJC Scopus subject areas

    • Immunology and Allergy
    • Immunology

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