PRINCIPAL
INVESTIGATORS

Principal Investigator

Bodo Grimbacher

Background

Prof. Bodo Grimbacher is a physician scientist and the Vice-Director of the Institute for Immunodeficiency at the University Hospital Freiburg. He also serves as an honorary consultant for Immunology at the Royal Free Hospital, University College London, where he led an EU-Marie-Curie team until 2011.

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Earlier, he was a senior scientist in Clinical Immunology (Freiburg) and completed a postdoc at the NIH’s National Human Genome Institute.

His accolades include the Emmy-Noether Fellowship (DFG), Marie-Curie Excellence Grant (EU), Georges Köhler Award (DGfI), Richard Farr Memorial Lectureship (AAAAI), Rudolf-Schoen Prize, and Leopoldina’s Thieme Prize.

In 2004, he launched the ESID registry, an EU-funded database for over 200 different primary immunodeficiencies, and served as Secretary of the European Society for Immunodeficiencies (2006–2010).

Prof. Grimbacher is a current member of the Scientific Advisory Board of the DADA2 Foundation, of the Scientific Advisory Board of the ACHSE e.V., he is CO-Speaker of the E-Rare Disease Network iPAD (EU), speaker of the German Rare Disease Network GAIN (BMBF) and speaker of the Translational School of Immunology (DGfI).

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Research

Grimbacher’s research group explores inborn errors of the immune system through a multidisciplinary approach, combining molecular and cellular biology, genetics, epigenetics, microbiome research, gene therapy, bioinformatics, and translational science. Their goal is to uncover disease mechanisms while advancing treatment options for affected patients.

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A key contributor to the field, Grimbacher has helped identify over 18 monogenic causes of primary immunodeficiencies, including the first genetic drivers of common variable immunodeficiency (the most widespread PID), severe congenital neutropenia (such as Kostmann syndrome), hyper-IgE syndromes, chronic mucocutaneous candidiasis, and inflammatory bowel disease (IL10/IL10-receptor deficiency).

His current team focuses on critical pathways like NF-κB signaling, T cell co-stimulation (particularly CTLA-4 and LRBA), autophagy in immunity, JAK-STAT signaling and IgE regulation, IL10’s role in inflammatory bowel disease, and host defense mechanisms against Candida.

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Publications

Rauer S, Marks R, Urbach H, Warnatz K, Nath A, Holland S, Weiller C, Grimbacher B. (2019) Treatment of Progressive Multifocal Leukoencephalopathy with Pembrolizumab. N Engl J Med. (letter) Apr 25;380(17):1676-1677.

Frey-Jakobs S, Hartberger JM, Fliegauf M, Bossen C, Wehmeyer ML, Neubauer JC, Bulashevska A, Proietti M, Fröbel P, Nöltner C, Yang L, Rojas-Restrepo J, Langer N, Winzer S, Engelhardt KR, Glocker C, Pfeifer D, Klein A, Schäffer AA, Lagovsky I, Lachover-Roth I, Béziat V, Puel A, Casanova JL, Fleckenstein B, Weidinger S, Kilic SS, Garty BZ, Etzioni A, Grimbacher B. (2018) ZNF341 controls STAT3 expression and thereby immunocompetence. Sci Immunol. 15;3(24).

Fliegauf M, Bryant VL, Frede N, Slade C, Woon ST, Lehnert K, Winzer S, Bulashevska A, Scerri T, Leung E, Jordan A, Keller B, de Vries E, Cao H, Yang F, Schäffer AA, Warnatz K, Browett P, Douglass J, Ameratunga RV, van der Meer JW, Grimbacher B. (2015) Haploinsufficiency of the NF-κB1 Subunit p50 in Common Variable Immunodeficiency. Am J Hum Genet. 97(3):389-403.

Schubert D, Bode C, Kenefeck R, Hou TZ, Wing JB, Kennedy A, Bulashevska A, Petersen BS, Schäffer AA, Grüning BA, Unger S, Frede N, Baumann U, Witte T, Schmidt RE, Dueckers G, Niehues T, Seneviratne S, Kanariou M, Speckmann C, Ehl S, Rensing-Ehl A, Warnatz K, Rakhmanov M, Thimme R, Hasselblatt P, Emmerich F, Cathomen T, Backofen R, Fisch P, Seidl M, May A, Schmitt-Graeff A, Ikemizu S, Salzer U, Franke A, Sakaguchi S, Walker LS*, Sansom DM*, Grimbacher B*. (2014) Autosomal dominant immune dysregulation syndrome in humans with CTLA4 mutations. Nat Med. 20(12):1410-6.

Glocker, E. O., D. Kotlarz, K. Boztug, E. M. Gertz, A. A. Schaffer, F. Noyan, M. Perro, J. Diestelhorst, A. Allroth, D. Murugan, N. Hatscher, D. Pfeifer, K. W. Sykora, M. Sauer, H. Kreipe, M. Lacher, R. Nustede, C. Woellner, U. Baumann, U. Salzer, S. Koletzko, N. Shah, A. W. Segal, A. Sauerbrey, S. Buderus, S. B. Snapper, B. Grimbacher, and C. Klein. (2009) Inflammatory bowel disease and mutations affecting the interleukin-10 receptor. N Engl J Med. 361: 2033-2045.