SFB 620:  Immunodeficiency: Clinical and Animal Models

• Subject Area: Medicine
• Term: from 2002 to 2013
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 5485303

During recent years molecular causes of primary human immunodeficiencies have been increasingly identified and in some of these conditions causal therapies have become feasible. Instrumental in this endeavour have been detailed phenotypic analyses of immunologically relevant cell types, progress in genome and linkage analysis and the exact knowledge of murine phenotypes with defined gene defects. These new developments have led to the foundation of the first German collaborative research centre devoted to Immunodeficiencies in July 2002 at Freiburg University. The program combines 13 research proposals from clinical and basic immunological backgrounds. Six projects (Section A) deal with animal models of immunodeficiency providing the knowledge for a better understanding and improved diagnosis of corresponding human diseases. Four projects (Section B) are devoted to overlapping features between animal models and clinical observations. Here findings from animal models may be directly applied to clinical problems. Finally in Section C three projects deal with clinical model diseases which not only provide the research topic but also the human cells and tissues for biologic and genetic analysis. The collaborative research centre 620 gathers proposals on B cells associated immunodeficiencies as wells as inherited and virus-induced T cell immunodeficiency models.

• DFG Programme: Collaborative Research Centres

Completed projects

• A01 - The role of SLP-65-induced FoxO transcription factors in B cell development (Project Head Jumaa, Hassan )
• A02 - Regulation der B Lymphotyten Selektion durch Transkriptionsfaktoren der Early Growth Response (Egr) Genfamilie (Project Head Eibel, Hermann )
• A04 - The impact of viral infections on the immunological and clinical phenotype of "leaky" SCID mice (Project Head Ehl, Stephan )
• A05 - Borna Disease Virus-induzierte neurologische Erkrankung der Maus: Selektiver Neuronenverlust bei Interferon-y-Defizienz (Project Head Stäheli, Peter )
• A07 - Untersuchung der frühesten Thymus-Einwanderer und ihrer Nische im Rhymus in der Maus als Grundlage des Verständnisses der Thymus-Repopulation nach myelo-ablativer Therapie im Menschen (Project Head Bleul, Conrad Christian )
• A08 - Genetische Analyse der Thymusentwicklung bei Vertebraten (Project Heads Boehm, Thomas ;  Schorpp, Marcel )
• A09 - Expression, Funktion und therapeutische Modulation der Arginase bei der akuten und chronischen Infektion mit Leishmania major (Project Heads Bogdan, Christian ;  Schleicher, Ulrike )
• A10 - Role of EBF transcription factors in normal and malignant lymphopoiesis (Project Head Grosschedl, Rudolf )
• A11 - Molecular mechanisms of thymus colonization: reconstruction of the thymic niche (Project Head Boehm, Thomas )
• A12 - Interferon response and virus resistance of mice with defects in the innate immune system (Project Head Stäheli, Peter )
• A13 - New faces of type I interferons: from immunosuppression to tissue homeostasis (Project Head Prinz, Marco )
• A14 - Analysis of CD8 T cell responses in immunodeficient mice lacking the NKG2D receptor (Project Head Diefenbach, Andreas )
• A15 - Control of graft-versus-host disease for enhanced immune reconstitution following allogeneic hematopoietic cell transplantation (Project Head Zeiser, Robert )
• A16 - Exploring the Role of microRNAs in the Immune System (Project Head Fukao, Taro )
• A17 - Immunodeficiencies due to neutrophil dysfunction: molecular analyses of signalling pathways (Project Head Häcker, Georg )
• B1 - Die Rolle des Transkriptionsfaktors BOB.1 in der B-Zellentwicklung (Project Heads Nielsen, Ph.D., Peter ;  Reth, Michael )
• B02 - Local silencing of lymphocyte function by cadherins through interaction with the inhibitory receptor KLRG1 (Project Head Pircher, Hanspeter )
• B3 - Die Bedeutung von Osteopontin (OPN) für Immundefizienz-Syndrome: Erkrankungen des atopischen Formenkreises und Hyper IgE Syndrom (HIES) (Project Head Simon, Jan-Christoph )
• B04 - Induzierbare Immundefizienz mittels HIV-1-Nef (Project Head Niedermann, Gabriele )
• B05 - Production and analysis of B-cell mutant mice with the Cre/loxP method (Project Head Reth, Michael )
• B06 - CD3y-deficiency in humans and mice (Project Head Schamel, Wolfgang )
• B07 - Untersuchungen zur Funktion der Inositol-5-Phosphatase SHIP in der Xid/XLA-Immundefizienz (Project Head Huber, Michael )
• B08 - Inflammatory signal induction and termination in chronic granulomatous (Project Head Henneke, Philipp )
• C01 - Disturbed peripheral B cell differentiation in patients with Common variable immunodeficiency (CVID) - origin and function of CD21low B cells. (Project Heads Peter, Hans-Hartmut ;  Warnatz, Klaus )
• C02 - Kopplungsanalysen beim familiären variablen Immundefekt (CVID) und selektiven IgA-Defekt - ein Weg zur Identifizierung der genetischen Defekte (Project Head Grimbacher, Bodo )
• C04 - Humane NK-Zell und T-Zell Subpopulationen bei primärer und sekundärer Immundefizienz (Project Head Fisch, Paul )
• C05 - Entzündliche Autoimmunkrankheiten der Haut: Molekulare Charakterisierung des Autoantigens Kollagen XVII/BP180 (Project Head Bruckner-Tuderman, Leena Kaarina )
• C06 - Identification and functional analysis of regulatory T cells during chronic HCV infection (Project Head Thimme, Robert )
• C07 - Analysis of microRNAs in normal human peripheral B-cell development and patients with primary antibody deficiencies (Project Head Salzer, Ulrich )
• C08 - Defining defects of pulmonary immuno-regulation in patients with common variable immunodeficiency (CVID) and sarcoidosis (Project Head Prasse, Antje )
• Z01 - Central tasks of the Collaborative Research Center (Project Head Pircher, Hanspeter )
• Z02 - Molecular and Cellular Analyses in Primary and Secondary Immunodeficiencies (Project Head Fisch, Paul )
• Z03 - Integrated Research Training Group of Immunodeficiency (IRTG-IMM) (Project Heads Diefenbach, Andreas ;  Schamel, Wolfgang )

• Applicant Institution: Albert-Ludwigs-Universität Freiburg
• Participating Institution: Max-Planck-Institut für Immunbiologie und Epigenetik; Universitätsklinikum Freiburg der Albert-Ludwigs-Universität Freiburg
• Spokesperson: Professor Dr. Hanspeter Pircher, since 7/2009