Bodo Grimbacher

Principal Investigator

Institute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI)
Medical Center – University of Freiburg

Breisacher Str. 115
79106 Freiburg i. Brsg.

Current position

Professor, Institute for Immunodeficiency, Center for Chronic Immunodeficiency,
Medical Center – University of Freiburg

Academic training

1988 – 1995 Study of Medicine in Aachen, Freiburg and Hamburg

Scientific qualifications

2006 Habilitation in Internal Medicine, University of Freiburg
(Mentor: Prof. H. H. Peter)
1995 Dissertation in Medicine, University of Freiburg
(Supervisor: Prof. H. Eibel)

Postgraduate Positions

2011 – 2018 W3 Professor, Scientific Director and consultant at the CCI, Medical Center - University of Freiburg
2006 – 2011 Consultant and Head of Marie-Curie Research Group, Dept. of Immunology, Royal Free Hospital & University College London, UK
2000 – 2006 Assistant lecturer and Emmy Noether-Fellow of the DFG,
Division of Rheumatology and Clinical Immunology,
Medical Center – University of Freiburg
1997 – 2000 Postdoctorate, NIH, National Human Genome Institute, Bethesda, MD, USA
1995 – 1997 Postdoctorate, Division of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg

Miscellaneous (Honors, Awards)

2009 Thieme Research Prize of the Leopoldina, Halle
2007 Rudolph-Schoen Prize, Hannover Medical School
2006 Marie-Curie Excellence Award of the European Commission
2006 Georges Köhler Award of the German Immunology Society
2002 Admission to the Emmy Noether Program of the German Research Foundation

link to all publications from B. Grimbacher: Pubmed

Publications based on CRC1160 funding

Harder, I., M. Munchhalfen, G. Andrieux, Boerries, B. Grimbacher, H. Eibel, M. E. Maccari, S. Ehl, J. Wienands, J. Jellusova, K. Warnatz, and B. Keller. 2022. Dysregulated PI3K Signaling in B Cells of CVID Patients. Cells 11:464. doi: 10.3390/cells11030464.

James AE, West L, Schloss K, Nataraj P, Urban A, Hirsch A, Krausz M, Kumar S, Raasch J, Risma K, Church JA, Grimbacher B, Bergerson JRE, Chong H, Freeman AF. 2022. Treatment of STAT3-deficient hyper-immunoglobulin E syndrome with monoclonal antibodies targeting allergic inflammation. J Allergy Clin Immunol Pract. Jan 24:S2213-2198(22)00027-7.

Sperlich JM, Grimbacher B, Soetedjo V, Workman S, Burns SO, Lowe DM, Hurst JR. 2022. Predictive Factors for and Complications of Bronchiectasis in Common Variable Immunodeficiency Disorders. J Clin Immunol. 2022 Jan 11. doi: 10.1007/s10875-022-01206-8.

Rojas-Restrepo J, Caballero-Oteyza A, Huebscher K, Haberstroh H, Fliegauf M, Keller B, Kobbe R, Warnatz K, Ehl S, Proietti M, Grimbacher B. 2021. Establishing the Molecular Diagnoses in a Cohort of 291 Patients With Predominantly Antibody Deficiency by Targeted Next-Generation Sequencing: Experience From a Monocentric Study. Front Immunol. 12:786516. doi: 10.3389/fimmu.2021.786516.

Yang L, Booth C, Speckmann C, Seidel MG, Worth AJ, Kindle G, Lankester AC, B G; ESID Clinical and Registry Working Parties, Gennery AR, Seppanen MR, Morris EC, Burns SO. Phenotype, genotype, treatment, and survival outcomes in patients with X-linked inhibitor of apoptosis deficiency.2021.  J Allergy Clin Immunol. S0091-6749(21)02597-5. doi: 10.1016/j.jaci.2021.10.037. Online ahead of print.

van Schewick CM, Lowe DM, Burns SO, Workman S, Symes A, Guzman D, Moreira F, Watkins J, Clark I, Grimbacher B. 2021. Bowel Histology of CVID Patients Reveals Distinct Patterns of Mucosal Inflammation. J Clin Immunol. doi: 10.1007/s10875-021-01104-5. Online ahead of print.

Haring E, Andrieux G, Uhl FM, Krausz M, Proietti M, Sauer B, Esser PR, Martin SF, Pfeifer D, Schmitt-Graeff A, Duyster J, Köhler N, Grimbacher B, Boerries M, Aumann K, Zeiser R, Apostolova P. 2021. Therapeutic targeting of endoplasmic reticulum stress in acute graft-versus-host disease. Haematologica. doi: 10.3324/haematol.2021.278387.

Salzer U, Grimbacher B. 2021. TACI deficiency – a complex system out of balance. Curr Opin Immunol. 71:81-88.

Volk T, Warnatz K, Marks R, Urbach H, Schluh G, Strohmeier V, Rojas-Restrepo J, Grimbacher B, Rauer S. 2021. Pembrolizumab for treatment of progressive multifocal leukoencephalopathy in primary immunodeficiency and/or hematologic malignancy: a case series of five patients. J Neurol. 2021 Jul 1. doi: 10.1007/s00415-021-10682-8.

Ramirez NJ, Posadas-Cantera S, Caballero-Oteyza A, Camacho-Ordonez N, Grimbacher B. 2021. There is no gene for CVID – novel monogenetic causes for primary antibody deficiency. Curr Opin Immunol. 2021 Jun 18;72:176-185.

Egg D, Rump IC, Mitsuiki N, Rojas-Restrepo J, Maccari ME, Schwab C, Gabrysch A, Warnatz K, Goldacker S, Patiño V, Wolff D, Okada S, Hayakawa S, Shikama Y, Kanda K, Imai K, Sotomatsu M, Kuwashima M, Kamiya T, Morio T, Matsumoto K, Mori T, Yoshimoto Y, Dybedal I, Kanariou M, Kucuk ZY, Chapdelaine H, Petruzelkova L, Lorenz HM, Sullivan KE, Heimall J, Moutschen M, Litzman J, Recher M, Albert MH, Hauck F, Seneviratne S, Pachlopnik Schmid J, Kolios A, Unglik G, Klemann C, Snapper S, Giulino-Roth L, Svaton M, Platt CD, Hambleton S, Neth O, Gosse G, Reinsch S, Holzinger D, Kim YJ, Bakhtiar S, Atschekzei F, Schmidt R, Sogkas G, Chandrakasan S, Rae W, Derfalvi B, Marquart HV, Ozen A, Kiykim A, Karakoc-Aydiner E, Králíčková P, de Bree G, Kiritsi D, Seidel MG, Kobbe R, Dantzer J, Alsina L, Armangue T, Lougaris V, Agyeman P, Nyström S, Buchbinder D, Arkwright PD, Grimbacher B. 2021. Therapeutic options for CTLA-4 insufficiency. J Allergy Clin Immunol. S0091-6749(21)00891-5.

Català-Moll F, Ferreté-Bonastre AG, Li T, Weichenhan D, Lutsik P, Ciudad L, Álvarez-Prado ÁF, Rodríguez-Ubreva J, Klemann C, Speckmann C, Vilas-Zornoza A, Abolhassani H, Martínez-Gallo M, Dieli-Crimi R, Rivière JG, Martín-Nalda A, Colobran R, Soler-Palacín P, Kracker S, Hammarström L, Prosper F, Durandy A, Grimbacher B, Plass C, Ballestar E. 2021. Activation-induced deaminase is critical for the establishment of DNA methylation patterns prior to the germinal center reaction. Nucleic Acids Res. 49(9):5057-5073.

Fliegauf M, Krüger R, Steiner S, Hanitsch LG, Büchel S, Wahn V, von Bernuth H, Grimbacher B. 2021. A Pathogenic Missense Variant in NFKB1 Causes Common Variable Immunodeficiency Due to Detrimental Protein Damage. Front Immunol. 12:621503.

Gámez-Díaz L, Grimbacher B. 2021. Immune checkpoint deficiencies and autoimmune lymphoproliferative syndromes. Biomed J. 19:S2319-4170(21)00038-X. doi: 10.1016/j.bj.2021.04.005.

Wang, L., D. Aschenbrenner, Z. Zeng, X. Cao, D. Mayr, M. Mehta, M. Capitani, N. Warner, J. Pan, L. Wang, Q. Li, T. Zuo, S. Cohen-Kedar, J. Lu, R. C. Ardy, D. J. Mulder, D. Dissanayake, K. Peng, Z. Huang, X. Li, Y. Wang, X. Wang, S. Li, S. Bullers, A. N. Gammage, K. Warnatz, A. I. Schiefer, G. Krivan, V. Goda, W. H. A. Kahr, M. Lemaire, C. Genomics England Research, C. Y. Lu, I. Siddiqui, M. G. Surette, D. Kotlarz, K. R. Engelhardt, H. R. Griffin, R. Rottapel, H. Decaluwe, R. M. Laxer, M. Proietti, S. Hambleton, S. Elcombe, C. H. Guo, B. Grimbacher, I. Dotan, S. C. Ng, S. A. Freeman, S. B. Snapper, C. Klein, K. Boztug, Y. Huang, D. Li, H. H. Uhlig, and A. M. Muise. 2021. Gain-of-function variants in SYK cause immune dysregulation and systemic inflammation in humans and mice. Nat Genet 53: 500-510.

Camacho-Ordonez N, Ballestar E, Timmers HTM, Grimbacher B. 2021. What can clinical immunology learn from inborn errors of epigenetic regulators? J Allergy Clin Immunol. 147(5):1602-1618. doi: 10.1016/j.jaci.2021.01.035. Epub 2021 Feb 17.

Harrison SC, Tsilifis C, Slatter MA, Nademi Z, Worth A, Veys P, Ponsford MJ, Jolles S, Al-Herz W, Flood T, Cant AJ, Doffinger R, Barcenas-Morales G, Carpenter B, Hough R, Haraldsson Á, Heimall J, Grimbacher B, Abinun M, Gennery AR. 2021. Hematopoietic Stem Cell Transplantation Resolves the Immune Deficit Associated with STAT3-Dominant-Negative Hyper-IgE Syndrome.  J Clin Immunol. doi: 10.1007/s10875-021-00971-2. Online ahead of print.

van Schewick CM, Nöltner C, Abel S, Burns SO, Workman S, Symes A, Guzman D, Proietti M, Bulashevska A, Moreira F, Soetedjo V, Lowe DM, Grimbacher B. 2020. Altered Microbiota, Impaired Quality of Life, Malabsorption, Infection, and Inflammation in CVID Patients With Diarrhoea. Front Immunol. 11:1654. doi: 10.3389/fimmu.2020.01654. eCollection 2020.

Haring E, Uhl FM, Andrieux G, Proietti M, Bulashevska A, Sauer B, Braun LM, de Vega Gomez E, Esser PR, Martin SF, Pfeifer D, Follo M, Schmitt-Graeff A, Buescher J, Duyster J, Grimbacher B, Boerries M, Pearce EL, Zeiser R, Apostolova P. Haematologica. 2020. Bile acids regulate intestinal antigen presentation and reduce graft-versus-host disease without impairing the graft-versus-leukemia effect. Jul 16:haematol.2019.242990. doi: 10.3324/haematol.2019.242990. Online ahead of print.

Wolf T, Jin W, Zoppi G, Vogel IA, Akhmedov M, Bleck CKE, Beltraminelli T, Rieckmann JC, Ramirez NJ, Benevento M, Notarbartolo S, Bumann D, Meissner F, Grimbacher B, Mann M, Lanzavecchia A, Sallusto F, Kwee I, Geiger R. 2020. Dynamics in protein translation sustaining T cell preparedness. Nat Immunol. 21(8):927-937. doi: 10.1038/s41590-020-0714-5. Epub 2020 Jul 6.

Norona, J., P. Apostolova, D. Schmidt, R. Ihlemann, N. Reischmann, G. Taylor, N. Kohler, J. de Heer, S. Heeg, G. Andrieux, B. A. Siranosian, A. Schmitt-Graeff, D. Pfeifer, A. Catalano, I. Frew, M. Proietti, B. Grimbacher, A. Bulashevska, A. S. Bhatt, T. Brummer, T. S. Clauditz, T. Zabelina, N. Kroeger, B. R. Blazar, M. Boerries, F. Ayuk, and R. Zeiser. 2020. Glucagon like peptide-2 for Intestinal stem cell and Paneth cell repair during graft-versus-host disease in mice and humans. Blood. 2020005957. doi: 10.1182/blood.2020005957. Online ahead of print.

Beziat, V., S. J. Tavernier, Y. H. Chen, C. S. Ma, M. Materna, A. Laurence, J. Staal, D. Aschenbrenner, L. Roels, L. Worley, K. Claes, L. Gartner, L. A. Kohn, M. De Bruyne, K. Schmitz-Abe, L. M. Charbonnier, S. Keles, J. Nammour, N. Vladikine, M. R. L. Maglorius Renkilaraj, Y. Seeleuthner, M. Migaud, J. Rosain, M. Jeljeli, B. Boisson, E. Van Braeckel, J. A. Rosenfeld, H. Dai, L. C. Burrage, D. R. Murdock, B. N. Lambrecht, V. Avettand-Fenoel, T. P. Vogel, N. Undiagnosed Diseases, C. R. Esther, S. Haskologlu, F. Dogu, P. Ciznar, D. Boutboul, M. Ouachee-Chardin, J. Amourette, M. N. Lebras, C. Gauvain, C. Tcherakian, A. Ikinciogullari, R. Beyaert, L. Abel, J. D. Milner, B. Grimbacher, L. J. Couderc, M. J. Butte, A. F. Freeman, E. Catherinot, C. Fieschi, T. A. Chatila, S. G. Tangye, H. H. Uhlig, F. Haerynck, J. L. Casanova, and A. Puel. 2020. Dominant-negative mutations in human IL6ST underlie hyper-IgE syndrome. J Exp Med 217. pii: e20191804. doi: 10.1084/jem.20191804.

Troilo, A., C. Wehr, I. Janowska, N. Venhoff, J. Thiel, J. Rawluk, N. Frede, J. Staniek, R. Lorenzetti, M. T. Schleyer, G. W. Herget, L. Konstantinidis, M. Erlacher, M. Proietti, N. Camacho-Ordonez, R. E. Voll, B. Grimbacher, K. Warnatz, U. Salzer, and M. Rizzi. 2020. Nonpermissive bone marrow environment impairs early B-cell development in common variable immunodeficiency. Blood 135: 1452-1457.

Cekic, S., J. M. Hartberger, S. Frey-Jakobs, H. Huriyet, M. B. Hortoglu, J. C. Neubauer, Y. Karali, C. D. Abakay, O. Saraydaroglu, T. Cavas, B. Grimbacher, and S. S. Kilic. 2020. Cancer Tendency in a Patient with ZNF341 Deficiency. J Clin Immunol. doi: 10.1007/s10875-020-00756-z. [Epub ahead of print]

Schröder C, Sogkas G, Fliegauf M, Dörk T, Liu D, Hanitsch LG, Steiner S, Scheibenbogen C, Jacobs R, Grimbacher B, Schmidt RE, Atschekzei F. 2019. Late-Onset Antibody Deficiency Due to Monoallelic Alterations in NFKB1. Front Immunol. 10:2618.

van de Ven A, Mader I, Wolff D, Goldacker S, Fuhrer H, Rauer S, Grimbacher B, Warnatz K. 2019. Structural non-infectious manifestations of the central nervous system in common variable immunodeficiency disorders. J Allergy Clin Immunol Pract. pii: S2213-2198(19)31026-8.

Dorjbal B, Stinson JR, Ma CA, Weinreich MA, Miraghazadeh B, Hartberger JM, Frey-Jakobs S, Weidinger S, Moebus L, Franke A, Schäffer AA, Bulashevska A, Fuchs S, Ehl S, Limaye S, Arkwright PD, Briggs TA, Langley C, Bethune C, Whyte AF, Alachkar H, Nejentsev S, DiMaggio T, Nelson CG, Stone KD, Nason M, Brittain EH, Oler AJ, Veltri DP, Leahy TR, Conlon N, Poli MC, Borzutzky A, Cohen JI, Davis J, Lambert MP, Romberg N, Sullivan KE, Paris K, Freeman AF, Lucas L, Chandrakasan S, Savic S, Hambleton S, Patel SY, Jordan MB, Theos A, Lebensburger J, Atkinson TP, Torgerson TR, Chinn IK, Milner JD, Grimbacher B, Cook MC, Snow AL. 2019. Hypomorphic caspase activation and recruitment domain 11 (CARD11) mutations associated with diverse immunologic phenotypes with or without atopic disease. J Allergy Clin Immunol. 143(4):1482-1495.

Vandrovcova J, Salzer U, Grimbacher B, Wanders J, Rao K, Thrasher A, Burns S, Gilmore K, Bussel J, Cooper N. 2019. FAS mutations are an uncommon cause of immune thrombocytopenia in children and adults without additional features of immunodeficiency. Br J Haematol. 186(6):e163-e165.

von Spee-Mayer, C., V. Koemm, C. Wehr, S. Goldacker, G. Kindle, A. Bulashevska, M. Proietti, Grimbacher, S. Ehl, and K. Warnatz. 2019. Evaluating laboratory criteria for combined immunodeficiency in adult patients diagnosed with common variable immunodeficiency. Clin Immunol. 203:59-62.

Eskandarian, Z., M. Fliegauf, A. Bulashevska, M. Proietti, R. Hague, C. R. Smulski, D. Schubert, Warnatz, and B. Grimbacher. 2019. Assessing the Functional Relevance of Variants in the IKAROS Family Zinc Finger Protein 1 (IKZF1) in a Cohort of Patients With Primary Immunodeficiency. Front Immunol 10: 568.

Mitsuiki, N., C. Schwab, and B. Grimbacher. 2019. What did we learn from CTLA-4 insufficiency on the human immune system? Immunol Rev 287: 33-49.

Klemann C, Camacho-Ordonez N, Yang L, Eskandarian Z, Rojas-Restrepo JL, Frede N, Bulashevska A, Heeg M, Al-Ddafari MS, Premm J, Seidl M, Ammann S, Sherkat R, Radhakrishnan N, Warnatz K, Unger S, Kobbe R, Hüfner A, Leahy TR, Ip W, Burns SO, Fliegauf M, Grimbacher B. 2019. Clinical and Immunological Phenotype of Patients With Primary Immunodeficiency Due to Damaging Mutations in NFKB2. Front Immunol. 10:297.

Stefanie Frey-Jakobs, Julia M. Hartberger, Manfred Fliegauf, Claudia Bossen, Magdalena L. Wehmeyer, Johanna C. Neubauer, Alla Bulashevska, Michele Proietti, Philipp Fröbel, Christina Nöltner, Linlin Yang, Jessica Rojas-Restrepo, Niko Langer, Sandra Winzer, Karin R. Engelhardt, Cristina Glocker, Dietmar Pfeifer, Adi Klein, Alejandro A. Schäffer, Irina Lagovsky, Idit Lachover-Roth, Vivien Béziat, Anne Puel, Jean-Laurent Casanova, Bernhard Fleckenstein, Stephan Weidinger, Sara S. Kilic, Ben-Zion Garty, Amos Etzioni, and Bodo Grimbacher. 2018. ZNF341 controls STAT3 expression and thereby immunocompetence. Sci Immunol.  15(24). pii: eaat4941.

Egg, D., C. Schwab, A. Gabrysch, P. D. Arkwright, E. Cheesman, L. Giulino-Roth, O. Neth, S. Snapper, S. Okada, M. Moutschen, P. Delvenne, A. C. Pecher, D. Wolff, Y. J. Kim, S. Seneviratne, K. M. Kim, J. M. Kang, S. Ojaimi, C. McLean, Warnatz, M. Seidl, and B. Grimbacher. 2018. Increased Risk for Malignancies in 131 Affected CTLA4 Mutation Carriers. Front Immunol 9: 2012.

Fliegauf, M., and Grimbacher. 2018. Nuclear factor kappaB mutations in human subjects: The devil is in the details. J Allergy Clin Immunol 142: 1062-1065.

Jung, S., L. Gamez-Diaz, M. Proietti, and Grimbacher. 2018. Immune TOR-opathies, a Novel Disease Entity in Clinical Immunology. Front Immunol 9: 966.

Schwab, C., A. Gabrysch, P. Olbrich, V. Patino, Warnatz, D. Wolff, A. Hoshino, M. Kobayashi, K. Imai, M. Takagi, I. Dybedal, J. A. Haddock, D. Sansom, J. M. Lucena, M. Seidl, A. Schmitt-Graff, V. Reiser, F. Emmerich, N. Frede, A. Bulashevska, U. Salzer, D. Schubert, S. Hayakawa, S. Okada, M. Kanariou, Z. Y. Kucuk, H. Chapdelaine, L. Petruzelkova, Z. Sumnik, A. Sediva, M. Slatter, P. D. Arkwright, A. Cant, H. M. Lorenz, T. Giese, V. Lougaris, A. Plebani, C. Price, K. E. Sullivan, M. Moutschen, J. Litzman, T. Freiberger, F. L. van de Veerdonk, M. Recher, M. H. Albert, F. Hauck, S. Seneviratne, J. P. Schmid, A. Kolios, G. Unglik, C. Klemann, C. Speckmann, S. Ehl, A. Leichtner, R. Blumberg, A. Franke, S. Snapper, S. Zeissig, C. Cunningham-Rundles, L. Giulino-Roth, O. Elemento, G. Duckers, T. Niehues, E. Fronkova, V. Kanderova, C. D. Platt, J. Chou, T. Chatila, R. Geha, E. McDermott, S. Bunn, M. Kurzai, A. Schulz, L. Alsina, F. Casals, A. Deya-Martinez, S. Hambleton, H. Kanegane, K. Tasken, O. Neth, and B. Grimbacher. 2018. Phenotype, penetrance, and treatment of 133 CTLA-4-insufficient individuals. J Allergy Clin Immunol. 142: 1932-1946.

Schmidt RE, Grimbacher B, Witte T. 2017. Autoimmunity and primary immunodeficiency: two sides of the same coin? Nat Rev Rheumatol. 14(1):7-18. Review.

Gamez-Diaz, L., J. Neumann, F. Jager, M. Proietti, F. Felber, P. Soulas-Sprauel, L. Perruzza, F. Grassi, T. Kogl, Aichele, M. Kilimann, B. Grimbacher, and S. Jung. 2017. Immunological phenotype of the murine Lrba knockout. Immunol Cell Biol 95: 789-802.