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Pemphigus - From autoimmunity to disease

Objective

Pemphigus is a potentially lethal bullous disease of the skin and mucosa and may be considered as a paradigmatic organ-specific autoimmune disease due to 1) its well-defined autoantigens, 2) the knowledge of critical events of its immune pathogenesis and 3) the reproducibility of major clinical and pathogenic features in suitable animal models. Overall, understanding the etiopathogenesis of pemphigus may provide crucial additional insight into basic mechanisms leading from autoimmunity to autoimmune disease and may help to design more specific therapeutic strategies. Despite the enormous progress, no specific therapy is currently available in pemphigus. The present project arises as a joined effort of European Scientists and Clinicians to establish a Consortium of groups engaged in providing a critical mass of patients and research tools to address the following goals: 1) to better define the immune pathogenesis of pemphigus utilizing two in vivo models of pemphigus with emphasis on autoaggressive T cells and their collaboration with autoantibody (autoAb) secreting B cells. 2) analysis of the autoAb-driven effector phase frequently involving “epitope spreading” which ultimately leads to a more severe disease; 3) characterization of the molecular events following Ab binding to the target autoantigens by defining autoAb-induced signalling events in epidermal keratinocytes; 4) spectrum of the autoimmune B cell response and the impact of therapeutic strategies on the cellular and humoral autoimmune response in pemphigus utilizing human monoclonal Ab which will be analysed for their fine specificity and genetically and functionally characterized; and 5) clinical read-out parameters for prospective studies which are urgently needed as valid parameters for the extent and activity of disease and which will be compared to serological markers and life quality assessment in pemphigus.

Call for proposal

FP7-HEALTH-2007-A
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Funding Scheme

CP-FP - Small or medium-scale focused research project

Coordinator

PHILIPPS UNIVERSITAET MARBURG
Address
Biegenstrasse 10
35037 Marburg
Germany
Activity type
Higher or Secondary Education Establishments
EU contribution
€ 492 000
Administrative Contact
Michael Hertl (Prof.)

Participants (6)

PROVINCIA ITALIANA DELLA CONGREGAZIONE DEI FIGLI DELLA IMMACOLATA CONCEZIONE
Italy
EU contribution
€ 377 400
Address
Via Della Luce 46
00153 Roma
Activity type
Research Organisations
Administrative Contact
Giovanna Zambruno (Dr.)
UNIVERSITAETSKLINIKUM FREIBURG
Germany
EU contribution
€ 278 000
Address
Hugstetter Strasse 49
79106 Freiburg
Activity type
Higher or Secondary Education Establishments
Administrative Contact
Jürgen Dreyer
UNIVERSITAET BERN
Switzerland
EU contribution
€ 814 000
Address
Hochschulstrasse 6
3012 Bern
Activity type
Higher or Secondary Education Establishments
Administrative Contact
Eliane Müller (Prof.)
EBERHARD KARLS UNIVERSITAET TUEBINGEN
Germany
EU contribution
€ 290 000
Address
Geschwister-scholl-platz
72074 Tuebingen
Activity type
Higher or Secondary Education Establishments
Administrative Contact
Martin Röcken (Prof.)
UNIVERSITE DE ROUEN NORMANDIE
France
EU contribution
€ 260 000
Address
Rue Thomas Becket 1 Mont Saint Aignan
76821 Mont Saint Aignan Cedex
Activity type
Higher or Secondary Education Establishments
Administrative Contact
Nathalie Leschelle (Dr.)
FONDAZIONE PER L ISTITUTO DI RICERCA IN BIOMEDICINA
Switzerland
EU contribution
€ 308 000
Address
Via Vincenzo Vela 6
6500 Bellinzona
Activity type
Research Organisations
Administrative Contact
Fosca Bognuda (Ms.)