Studying inborn errors of immunity affecting neutrophil granulocytes DE
The recruitment and host defence functions of neutrophil granulocytes critically depend on the tightly controlled reorganization of the actin network. In our discovery pipeline for children with inborn errors of immunity, we recently identified novel genetic variants in three actin-regulatory genes: FSCN, MIGFILIN, and ARHGEF6. The clinical phenotypes are variable, ranging from neutropenia to a hyper-inflammatory syndrome. In the present proposal we aim to identify the molecular and cellular pathophysiological mechanisms underlying these newly defined inborn errors of immunity.
Team
Publications
Gasdermin D drives focal Crystalline Thrombotic Microangiopathy by accelerating Immunothrombosis and NecroinflammationWatanabe-Kusunoki, K.; Li, C.; Bandeira Honda, T. S.; Zhao, D.; Kusunoki, Y.; Ku, J.; Long, H.; Klaus, M.; Han, C.; Braun, A.; Mammadova-Bach, E.; Linkermann, A.; Van Avondt, K.; Richter, M.; Soehnlein, O.; Linder, M. I.; Klein, C.; Steiger, S.; Anders, H.-J.
2024 Blood
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Generation of Complex Bone Marrow Organoids from Human Induced Pluripotent Stem Cells.Frenz-Wiessner, S.; Fairley, S. D.; Buser, M.; Goek, I.; Salewskij, K.; Jonsson, G.; Illig, D.; zu Putlitz, B.; Petersheim, D.; Li, Y.; Chen, P.-H.; Kalauz, M.; Conca, R.; Sterr, M.; Geuder, J.; Mizoguchi, Y.; Megens, R. T. A.; Linder, M. I.; Kotlarz, D.; Rudelius, M.; Penninger, J. M.; Marr, C.; Klein, C.
2024 Nat Methods
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Neutrophils - Biology and DiversityMaier-Begandt, D.; Alonso-Gonzalez, N.; Klotz, L.; Erpenbeck, L.; Jablonska, J.; Immler, R.; Hasenberg, A.; Mueller, T. T.; Herrero-Cervera, A.; Aranda-Pardos, I.; Flora, K.; Zarbock, A.; Brandau, S.; Schulz, C.; Soehnlein, O.; Steiger, S.; the TRR332 consortium.
2023 Nephrology Dialysis Transplantation
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Analyzing Mitochondrial Respiration of Human Induced Pluripotent Stem Cell-Derived Myeloid Progenitors Using Seahorse TechnologyFan, Y.; Mizoguchi, Y.; Tatematsu, M.; Linder, M. I.; Frenz, S.; Choi, J.; Klein, C.
2023 STAR Protoc
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Human Genetic Defects in SRP19 and SRPRA Cause Severe Congenital Neutropenia with Distinctive Proteome ChangesLinder, M. I.; Mizoguchi, Y.; Hesse, S.; Csaba, G.; Tatematsu, M.; Łyszkiewicz, M.; Zietara, N.; Jeske, T.; Hastreiter, M.; Rohlfs, M.; Liu, Y.; Grabowski, P.; Ahomaa, K.; Maier-Begandt, D.; Schwestka, M.; Pazhakh, V.; Isiaku, A.; Briones Miranda, B.; Blombery, P.; Saito, M. K.; Rusha, E.; Alizadeh, Z.; Pourpak, Z.; Kobayashi, M.; Rezaei, N.; Unal, E.; Hauck, F.; Drukker, M.; Walzog, B.; Rappsilber, J.; Zimmer, R.; Lieschke, G. J.; Klein, C.
2023 Blood
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