Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunity

Abstract Autoantibodies against the enzyme transglutaminase 3 (TG3) are characteristic to the gluten-sensitive skin disorder dermatitis herpetiformis (DH), which is an extraintestinal manifestation of celiac disease. We here demonstrate that TG3-specific B cells can activate gluten-specific CD4+ T c...

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Main Authors: Rasmus Iversen, Julie Elisabeth Heggelund, Saykat Das, Lene S. Høydahl, Ludvig M. Sollid
Format: Article
Language:English
Published: Nature Portfolio 2025-03-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-025-57564-5
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author Rasmus Iversen
Julie Elisabeth Heggelund
Saykat Das
Lene S. Høydahl
Ludvig M. Sollid
author_facet Rasmus Iversen
Julie Elisabeth Heggelund
Saykat Das
Lene S. Høydahl
Ludvig M. Sollid
author_sort Rasmus Iversen
collection DOAJ
description Abstract Autoantibodies against the enzyme transglutaminase 3 (TG3) are characteristic to the gluten-sensitive skin disorder dermatitis herpetiformis (DH), which is an extraintestinal manifestation of celiac disease. We here demonstrate that TG3-specific B cells can activate gluten-specific CD4+ T cells through B-cell receptor (BCR)-mediated internalization of TG3-gluten enzyme-substrate complexes. Stereotypic anti-TG3 antibodies using IGHV2-5/IGKV4-1 gene segments enhance the catalytic activity of TG3, and this effect translates into increased gluten presentation to T cells when such antibodies are expressed as BCRs. The crystal structure of TG3 bound to an IGHV2-5/IGKV4-1 Fab shows that antibody binding to a β-sheet in the catalytic core domain causes the enzyme to adopt the active conformation. This mechanism explains the production of stereotypic anti-TG3 autoantibodies in DH and highlights a role for TG3-specific B cells as antigen-presenting cells for gluten-specific T cells. Similar boosting effects of autoreactive BCRs could be relevant for other autoimmune diseases, including rheumatoid arthritis.
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spelling doaj-art-dc723d01ff4c4ac380070ac8563b93b22025-08-20T02:56:12ZengNature PortfolioNature Communications2041-17232025-03-0116111110.1038/s41467-025-57564-5Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunityRasmus Iversen0Julie Elisabeth Heggelund1Saykat Das2Lene S. Høydahl3Ludvig M. Sollid4Norwegian Coeliac Disease Research Centre, Institute of Clinical Medicine, University of OsloNorwegian Coeliac Disease Research Centre, Institute of Clinical Medicine, University of OsloNorwegian Coeliac Disease Research Centre, Institute of Clinical Medicine, University of OsloNorwegian Coeliac Disease Research Centre, Institute of Clinical Medicine, University of OsloNorwegian Coeliac Disease Research Centre, Institute of Clinical Medicine, University of OsloAbstract Autoantibodies against the enzyme transglutaminase 3 (TG3) are characteristic to the gluten-sensitive skin disorder dermatitis herpetiformis (DH), which is an extraintestinal manifestation of celiac disease. We here demonstrate that TG3-specific B cells can activate gluten-specific CD4+ T cells through B-cell receptor (BCR)-mediated internalization of TG3-gluten enzyme-substrate complexes. Stereotypic anti-TG3 antibodies using IGHV2-5/IGKV4-1 gene segments enhance the catalytic activity of TG3, and this effect translates into increased gluten presentation to T cells when such antibodies are expressed as BCRs. The crystal structure of TG3 bound to an IGHV2-5/IGKV4-1 Fab shows that antibody binding to a β-sheet in the catalytic core domain causes the enzyme to adopt the active conformation. This mechanism explains the production of stereotypic anti-TG3 autoantibodies in DH and highlights a role for TG3-specific B cells as antigen-presenting cells for gluten-specific T cells. Similar boosting effects of autoreactive BCRs could be relevant for other autoimmune diseases, including rheumatoid arthritis.https://doi.org/10.1038/s41467-025-57564-5
spellingShingle Rasmus Iversen
Julie Elisabeth Heggelund
Saykat Das
Lene S. Høydahl
Ludvig M. Sollid
Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunity
Nature Communications
title Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunity
title_full Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunity
title_fullStr Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunity
title_full_unstemmed Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunity
title_short Enzyme-activating B-cell receptors boost antigen presentation to pathogenic T cells in gluten-sensitive autoimmunity
title_sort enzyme activating b cell receptors boost antigen presentation to pathogenic t cells in gluten sensitive autoimmunity
url https://doi.org/10.1038/s41467-025-57564-5
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