Autoantibody Repertoire in APECED Patients Targets Two Distinct Subgroups of Protiens

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Fishman , D , Kisand , K , Hertel , C , Rothe , M , Remm , A , Pihlap , M , Adler , P , Vilo , J , Peet , A , Meloni , A , Podkrajsek , K T , Battelino , T , Bruserud , O , Wolff , A S B , Husebye , E S , Kluger , N , Krohn , K , Ranki , A , Peterson , H , Hayday , A & Peterson , P 2017 , ' Autoantibody Repertoire in APECED Patients Targets Two Distinct Subgroups of Protiens ' , Frontiers in Immunology , vol. 8 , 976 . https://doi.org/10.3389/fimmu.2017.00976

Title: Autoantibody Repertoire in APECED Patients Targets Two Distinct Subgroups of Protiens
Author: Fishman, Dmytro; Kisand, Kai; Hertel, Christina; Rothe, Mike; Remm, Anu; Pihlap, Maire; Adler, Priit; Vilo, Jaak; Peet, Aleksandr; Meloni, Antonella; Podkrajsek, Katarina Trebusak; Battelino, Tadej; Bruserud, Oyvind; Wolff, Anette S. B.; Husebye, Eystein S.; Kluger, Nicolas; Krohn, Kai; Ranki, Annamari; Peterson, Hedi; Hayday, Adrian; Peterson, Part
Contributor: University of Helsinki, Department of Dermatology, Allergology and Venereology
University of Helsinki, Clinicum
University of Helsinki, University of Helsinki
Date: 2017-08-16
Language: eng
Number of pages: 15
Belongs to series: Frontiers in Immunology
ISSN: 1664-3224
URI: http://hdl.handle.net/10138/224208
Abstract: High titer autoantibodies produced by B lymphocytes are clinically important features of many common autoimmune diseases. APECED patients with deficient autoimmune regulator (AIRE) gene collectively display a broad repertoire of high titer autoantibodies, including some which are pathognomonic for major autoimmune diseases. AIRE deficiency severely reduces thymic expression of gene-products ordinarily restricted to discrete peripheral tissues, and developing T cells reactive to those gene-products are not inactivated during their development. However, the extent of the autoantibody repertoire in APECED and its relation to thymic expression of self-antigens are unclear. We here undertook a broad protein array approach to assess autoantibody repertoire in APECED patients. Our results show that in addition to shared autoantigen reactivities, APECED patients display high inter-individual variation in their autoantigen profiles, which collectively are enriched in evolutionarily conserved, cytosolic and nuclear phosphoproteins. The APECED autoantigens have two major origins; proteins expressed in thymic medullary epithelial cells and proteins expressed in lymphoid cells. These findings support the hypothesis that specific protein properties strongly contribute to the etiology of B cell autoimmunity.
Subject: autoimmune regulator
autoantibodies
immune tolerance
thymus
autoantigen
SYNDROME TYPE-I
ANTI-CYTOKINE AUTOANTIBODIES
CHRONIC MUCOCUTANEOUS CANDIDIASIS
AMINO-ACID DECARBOXYLASE
AUTOIMMUNE REGULATOR
CELL TOLERANCE
SELF-TOLERANCE
HUMAN THYMUS
APS-I
AIRE
3121 General medicine, internal medicine and other clinical medicine
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