Nature Communications (Dec 2019)
CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome
- Catharina C. Gross,
- Céline Meyer,
- Urvashi Bhatia,
- Lidia Yshii,
- Ilka Kleffner,
- Jan Bauer,
- Anna R. Tröscher,
- Andreas Schulte-Mecklenbeck,
- Sebastian Herich,
- Tilman Schneider-Hohendorf,
- Henrike Plate,
- Tanja Kuhlmann,
- Markus Schwaninger,
- Wolfgang Brück,
- Marc Pawlitzki,
- David-Axel Laplaud,
- Delphine Loussouarn,
- John Parratt,
- Michael Barnett,
- Michael E. Buckland,
- Todd A. Hardy,
- Stephen W. Reddel,
- Marius Ringelstein,
- Jan Dörr,
- Brigitte Wildemann,
- Markus Kraemer,
- Hans Lassmann,
- Romana Höftberger,
- Eduardo Beltrán,
- Klaus Dornmair,
- Nicholas Schwab,
- Luisa Klotz,
- Sven G. Meuth,
- Guillaume Martin-Blondel,
- Heinz Wiendl,
- Roland Liblau
Affiliations
- Catharina C. Gross
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Céline Meyer
- Centre de Physiopathologie Toulouse-Purpan (CPTP), Université de Toulouse, CNRS, Inserm, UPS, CHU Purpan – BP 3028 – 31024
- Urvashi Bhatia
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Lidia Yshii
- Centre de Physiopathologie Toulouse-Purpan (CPTP), Université de Toulouse, CNRS, Inserm, UPS, CHU Purpan – BP 3028 – 31024
- Ilka Kleffner
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Jan Bauer
- Department of Neuroimmunology, Center for Brain Research, Medical University of Vienna
- Anna R. Tröscher
- Department of Neuroimmunology, Center for Brain Research, Medical University of Vienna
- Andreas Schulte-Mecklenbeck
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Sebastian Herich
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Tilman Schneider-Hohendorf
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Henrike Plate
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Tanja Kuhlmann
- Institute of Neuropathology, University Hospital Münster, University of Münster
- Markus Schwaninger
- Institute of Experimental and Clinical Pharmacology and Toxicology, University of Lübeck
- Wolfgang Brück
- Institute of Neuropathology, University Medical Center Göttingen
- Marc Pawlitzki
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- David-Axel Laplaud
- UMR 1064, INSERM, Centre de Recherche en Transplantation et Immunologie, Université de Nantes, CHU Nantes - Hôtel Dieu Bd Jean Monnet
- Delphine Loussouarn
- Service d’Anatomo-Pathologie, CHU Nantes, Hôtel-Dieu, rez-de-jardin
- John Parratt
- Department of Neurology, Royal North Shore Hospital
- Michael Barnett
- Brain and Mind Centre, Medical Faculty, University of Sydney
- Michael E. Buckland
- Brain and Mind Centre, Medical Faculty, University of Sydney
- Todd A. Hardy
- Brain and Mind Centre, Medical Faculty, University of Sydney
- Stephen W. Reddel
- Brain and Mind Centre, Medical Faculty, University of Sydney
- Marius Ringelstein
- Department of Neurology, Medical Faculty, Heinrich Heine University
- Jan Dörr
- Max Delbrueck Center for Molecular Medicine and Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, NeuroCure, Experimental and Clinical Research Center
- Brigitte Wildemann
- Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg
- Markus Kraemer
- Department of Neurology, Medical Faculty, Heinrich Heine University
- Hans Lassmann
- Department of Neuroimmunology, Center for Brain Research, Medical University of Vienna
- Romana Höftberger
- Institute of Neurology, Medical University of Vienna
- Eduardo Beltrán
- Institute of Clinical Neuroimmunology, Biomedical Center and Hospital of the Ludwig-Maximilians-University Munich
- Klaus Dornmair
- Institute of Clinical Neuroimmunology, Biomedical Center and Hospital of the Ludwig-Maximilians-University Munich
- Nicholas Schwab
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Luisa Klotz
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Sven G. Meuth
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Guillaume Martin-Blondel
- Centre de Physiopathologie Toulouse-Purpan (CPTP), Université de Toulouse, CNRS, Inserm, UPS, CHU Purpan – BP 3028 – 31024
- Heinz Wiendl
- Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster
- Roland Liblau
- Centre de Physiopathologie Toulouse-Purpan (CPTP), Université de Toulouse, CNRS, Inserm, UPS, CHU Purpan – BP 3028 – 31024
- DOI
- https://doi.org/10.1038/s41467-019-13593-5
- Journal volume & issue
-
Vol. 10,
no. 1
pp. 1 – 19
Abstract
Susac syndrome is an inflammatory pathology of the brain endothelium. Here the authors show that the pathology is driven by CD8 T cells attacking the endothelium, and that blocking T cell-endothelial adhesion ameliorates the disease in a mouse model, and associates with improved clinical score in 4 patients.
