Aire deficient mice do not develop the same profile of tissue-specific autoantibodies as APECED patients

Pöntynen, Nora (author): Dept of Molecular Medicine National Public Health Institute, Helsinki, Finland

Miettinen, Aaro (author): Dept of Bacteriology and Immunology University of Helsinki, Finland

Arstila, T. Petteri (author): Dept of Bacteriology and Immunology, University of Helsinki, Finland

Kämpe, Olle (author): Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe),Dept of Medical Sciences University of Uppsala, Sweden

Alimohammadi, Mohammad (author): Uppsala universitet,Institutionen för medicinska vetenskaper,Autoimmuna sjukdomar (Kämpe),Dept of Medical Sciences University of Uppsala, Sweden

Peltonen, Leena (author): Dept of Molecular Medicine National Public Health Institute, Helsinki, Finland

Ulmanen, Ismo (author): Dept of Molecular Medicine National Public Health Institute, Helsinki, Finland

(creator_code:org_t)

Elsevier BV, 2006
2006
English.
In: Journal of Autoimmunity. - : Elsevier BV. - 0896-8411 .- 1095-9157. ; 27:2, s. 96-104

Related links:: https://urn.kb.se/re...; show more...; https://doi.org/10.1...; https://urn.kb.se/re...; show less...

Abstract Subject headings

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED, or APS1), is a monogenic autoimmune disease caused by mutations in the autoimmune regulator (AIRE) gene. The three main components of APECED are chronic mucocuteaneous candidiasis, hypoparathyroidism and adrenocortical insufficiency. However, several additional endocrine or other autoimmune disease components, or ectodermal dystrophies form the individually variable clinical picture of APECED. An important feature of APECED is a spectrum of well-characterized circulating autoantibodies, reacting against tissue-specific autoantigens. Aire deficient mice develop some characteristics of APECED phenotype. In order to investigate whether the Aire deficient mice produce autoantibodies similar to human APECED, we studied the reactivity of Aire mouse sera against mouse homologues of 11 human APECED antigens. None of the APECED antigens indicated elevated reactivity in the Aire knock-out mouse sera, implying the absence of APECED associated autoantibodies in Aire deficient mice. These findings were supported by the failure of the autoantigens to activate mouse T-cells. Furthermore, Aire knock-out mice did not express increased levels of anti-nuclear antibodies compared to wt mice. This study indicates that spontaneous induction of tissue-specific autoantibodies similar to APECED does not occur in the rodent model suggesting differences in the immunopathogenic mechanisms between mice and men.

By the author/editor: Pöntynen, Nora; Miettinen, Aaro; Arstila, T. Pett ...; Kämpe, Olle; Alimohammadi, Mo ...; Vaarala, Outi, 1 ...; show more...; Peltonen, Leena; Ulmanen, Ismo; show less...

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