Search Results - "Häffner, Karsten"

CCDC103 mutations cause primary ciliary dyskinesia by disrupting assembly of ciliary dynein arms by Panizzi, Jennifer R, Becker-Heck, Anita, Castleman, Victoria H, Al-Mutairi, Dalal A, Liu, Yan, Loges, Niki T, Pathak, Narendra, Austin-Tse, Christina, Sheridan, Eamonn, Schmidts, Miriam, Olbrich, Heike, Werner, Claudius, Häffner, Karsten, Hellman, Nathan, Chodhari, Rahul, Gupta, Amar, Kramer-Zucker, Albrecht, Olale, Felix, Burdine, Rebecca D, Schier, Alexander F, O'Callaghan, Christopher, Chung, Eddie M K, Reinhardt, Richard, Mitchison, Hannah M, King, Stephen M, Omran, Heymut, Drummond, Iain A

“…Iain Drummond, Heymut Omran, Stephen King and colleagues show that CCDC103 mutations cause primary ciliary dyskinesia. Their studies suggest that CCDC103 is a…”
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Ciliary beat pattern and frequency in genetic variants of primary ciliary dyskinesia by RAIDT, Johanna, WALLMEIER, Julia, WERNER, Claudius, HJEIJ, Rim, ONNEBRINK, Jörg Grosse, PENNEKAMP, Petra, LOGES, Niki T, OLBRICH, Heike, HÄFFNER, Karsten, DOUGHERTY, Gerard W, OMRAN, Heymut

“…Primary ciliary dyskinesia (PCD) is a rare genetic disorder leading to recurrent respiratory tract infections. High-speed video-microscopy analysis (HVMA) of…”
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Multisystem inflammation and susceptibility to viral infections in human ZNFX1 deficiency by Vavassori, Stefano, Chou, Janet, Faletti, Laura Eva, Haunerdinger, Veronika, Opitz, Lennart, Joset, Pascal, Fraser, Christopher J., Prader, Seraina, Gao, Xianfei, Schuch, Luise A., Wagner, Matias, Hoefele, Julia, Maccari, Maria Elena, Zhu, Ying, Elakis, George, Gabbett, Michael T., Forstner, Maria, Omran, Heymut, Kaiser, Thomas, Kessler, Christina, Olbrich, Heike, Frosk, Patrick, Almutairi, Abduarahman, Platt, Craig D., Elkins, Megan, Weeks, Sabrina, Rubin, Tamar, Planas, Raquel, Marchetti, Tommaso, Koovely, Danil, Klämbt, Verena, Soliman, Neveen A., von Hardenberg, Sandra, Klemann, Christian, Baumann, Ulrich, Lenz, Dominic, Klein-Franke, Andreas, Schwemmle, Martin, Huber, Michael, Sturm, Ekkehard, Hartleif, Steffen, Häffner, Karsten, Gimpel, Charlotte, Brotschi, Barbara, Laube, Guido, Güngör, Tayfun, Buckley, Michael F., Kottke, Raimund, Staufner, Christian, Hildebrandt, Friedhelm, Reu-Hofer, Simone, Moll, Solange, Weber, Achim, Kaur, Hundeep, Ehl, Stephan, Hiller, Sebastian, Geha, Raif, Roscioli, Tony, Griese, Matthias, Pachlopnik Schmid, Jana

“…Recognition of viral nucleic acids is one of the primary triggers for a type I interferon–mediated antiviral immune response. Inborn errors of type I…”
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DNAH11 Localization in the Proximal Region of Respiratory Cilia Defines Distinct Outer Dynein Arm Complexes by Dougherty, Gerard W, Loges, Niki T, Klinkenbusch, Judith A, Olbrich, Heike, Pennekamp, Petra, Menchen, Tabea, Raidt, Johanna, Wallmeier, Julia, Werner, Claudius, Westermann, Cordula, Ruckert, Christian, Mirra, Virginia, Hjeij, Rim, Memari, Yasin, Durbin, Richard, Kolb-Kokocinski, Anja, Praveen, Kavita, Kashef, Mohammad A, Kashef, Sara, Eghtedari, Fardin, Häffner, Karsten, Valmari, Pekka, Baktai, György, Aviram, Micha, Bentur, Lea, Amirav, Israel, Davis, Erica E, Katsanis, Nicholas, Brueckner, Martina, Shaposhnykov, Artem, Pigino, Gaia, Dworniczak, Bernd, Omran, Heymut

“…Primary ciliary dyskinesia (PCD) is a recessively inherited disease that leads to chronic respiratory disorders owing to impaired mucociliary clearance…”
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Treating C3 glomerulopathy with eculizumab by Welte, Thomas, Arnold, Frederic, Kappes, Julia, Seidl, Maximilian, Häffner, Karsten, Bergmann, Carsten, Walz, Gerd, Neumann-Haefelin, Elke

“…C3 glomerulopathy (C3G) is a rare, but severe glomerular disease with grim prognosis. The complex pathogenesis is just unfolding, and involves acquired as well…”
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Impact of neutrophil extracellular traps on fluid properties, blood flow and complement activation by Burmeister, Antonia, Vidal-Y-Sy, Sabine, Liu, Xiaobo, Mess, Christian, Wang, Yuanyuan, Konwar, Swagata, Tschongov, Todor, Häffner, Karsten, Huck, Volker, Schneider, Stefan W, Gorzelanny, Christian

“…The intravascular formation of neutrophil extracellular traps (NETs) is a trigger for coagulation and blood vessel occlusion. NETs are released from…”
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Moss-produced human complement factor H with modified glycans has an extended half-life and improved biological activity by Tschongov, Todor, Konwar, Swagata, Busch, Andreas, Sievert, Christian, Hartmann, Andrea, Noris, Marina, Gastoldi, Sara, Aiello, Sistiana, Schaaf, Andreas, Panse, Jens, Zipfel, Peter F, Dabrowska-Schlepp, Paulina, Häffner, Karsten

“…Most drugs that target the complement system are designed to inhibit the complement pathway at either the proximal or terminal levels. The use of a natural…”
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Deletions and Point Mutations of LRRC50 Cause Primary Ciliary Dyskinesia Due to Dynein Arm Defects by Loges, Niki Tomas, Olbrich, Heike, Becker-Heck, Anita, Häffner, Karsten, Heer, Angelina, Reinhard, Christina, Schmidts, Miriam, Kispert, Andreas, Zariwala, Maimoona A., Leigh, Margaret W., Knowles, Michael R., Zentgraf, Hanswalter, Seithe, Horst, Nürnberg, Gudrun, Nürnberg, Peter, Reinhardt, Richard, Omran, Heymut

“…Genetic defects affecting motility of cilia and flagella cause chronic destructive airway disease, randomization of left-right body asymmetry, and, frequently,…”
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Different approaches to long-term treatment of aHUS due to MCP mutations: a multicenter analysis by Klämbt, Verena, Gimpel, Charlotte, Bald, Martin, Gerken, Christopher, Billing, Heiko, Loos, Sebastian, Hansen, Matthias, König, Jens, Vinke, Tobias, Montoya, Carmen, Lange-Sperandio, Bärbel, Kirschstein, Martin, Hennies, Imke, Pohl, Martin, Häffner, Karsten

“…Background Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening microangiopathy, frequently causing kidney failure. Inhibition of the terminal…”
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Correction to: Different approaches to long-term treatment of aHUS due to MCP mutations: a multicenter analysis by Klämbt, Verena, Gimpel, Charlotte, Bald, Martin, Gerken, Christopher, Billing, Heiko, Loos, Sebastian, Hansen, Matthias, König, Jens, Vinke, Tobias, Montoya, Carmen, Lange-Sperandio, Bärbel, Kirschstein, Martin, Hennies, Imke, Pohl, Martin, Häffner, Karsten

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Mutations in WDR19 encoding the intraflagellar transport component IFT144 cause a broad spectrum of ciliopathies by Fehrenbach, Henry, Decker, Christian, Eisenberger, Tobias, Frank, Valeska, Hampel, Tobias, Walden, Ulrike, Amann, Kerstin U., Krüger-Stollfuß, Ingrid, Bolz, Hanno J., Häffner, Karsten, Pohl, Martin, Bergmann, Carsten

“…Background An emerging number of clinically and genetically heterogeneous diseases now collectively termed ciliopathies have been connected to the dysfunction…”
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Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry by Omran, Heymut, Olbrich, Heike, Häffner, Karsten, Kispert, Andreas, Völkel, Alexander, Volz, Andreas, Sasmaz, Gürsel, Reinhardt, Richard, Hennig, Steffen, Lehrach, Hans, Konietzko, Nikolaus, Zariwala, Maimoona, Noone, Peadar G, Knowles, Michael, Mitchison, Hannah M, Meeks, Maggie, Chung, Eddie M.K, Hildebrandt, Friedhelm, Sudbrak, Ralf

“…Primary ciliary dyskinesia (PCD, MIM 242650) is characterized by recurrent infections of the respiratory tract due to reduced mucociliary clearance and by…”
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A synthetic protein as efficient multitarget regulator against complement over-activation by Ruiz-Molina, Natalia, Parsons, Juliana, Müller, Madeleine, Hoernstein, Sebastian N. W., Bohlender, Lennard L., Pumple, Steffen, Zipfel, Peter F., Häffner, Karsten, Reski, Ralf, Decker, Eva L.

“…The complement system constitutes the innate defense against pathogens. Its dysregulation leads to diseases and is a critical determinant in many viral…”
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Recombinant Production of MFHR1, A Novel Synthetic Multitarget Complement Inhibitor, in Moss Bioreactors by Top, Oguz, Parsons, Juliana, Bohlender, Lennard L, Michelfelder, Stefan, Kopp, Phillipp, Busch-Steenberg, Christian, Hoernstein, Sebastian N W, Zipfel, Peter F, Häffner, Karsten, Reski, Ralf, Decker, Eva L

“…The human complement system is an important part of the immune system responsible for lysis and elimination of invading microorganisms and apoptotic body…”
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Neutrophils activated by membrane attack complexes increase the permeability of melanoma blood vessels by Liu, Xiaobo, Wang, Yuanyuan, Bauer, Alexander T., Kirschfink, Michael, Ding, Peipei, Gebhardt, Christoffer, Borsig, Lubor, Tüting, Thomas, Renné, Thomas, Häffner, Karsten, Hu, Weiguo, Schneider, Stefan W., Gorzelanny, Christian

“…The microenvironment of malignant melanomas defines the properties of tumor blood vessels and regulates infiltration and vascular dissemination of immune and…”
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Homozygosity Mapping of a Gene Locus for Primary Ciliary Dyskinesia on Chromosome 5p and Identification of the Heavy Dynein Chain DNAH5 as a Candidate Gene by Omran, Heymut, Haffner, Karsten, Volkel, Alexander, Kuehr, Joachim, Ketelsen, Uwe-Peter, Ross, Uwe-Henning, Konietzko, Nikolaus, Wienker, Thomas, Brandis, Matthias, Hildebrandt, Friedhelm

“…Reduced mucociliary clearance in primary ciliary dyskinesia (PCD) causes recurrent infections of the upper and lower respiratory tract. The disease is usually…”
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Identification of a New Gene Locus for Adolescent Nephronophthisis, on Chromosome 3q22 in a Large Venezuelan Pedigree by Omran, Heymut, Fernandez, Carmen, Jung, Martin, Häffner, Karsten, Fargier, Bernardo, Villaquiran, Aminta, Waldherr, Rüdiger, Gretz, Norbert, Brandis, Matthias, Rüschendorf, Franz, Reis, André, Hildebrandt, Friedhelm

“…Nephronophthisis, an autosomal-recessive cystic kidney disease, is the most frequent monogenic cause for renal failure in childhood. Infantile and juvenile…”
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Revealing Complement Regulatory Functions of Thrombospondin-1 as New Potential Mechanism in Renal Disease: SA-PO592 by Konwar, Swagata, Kleindienst, Jessika, Decker, Eva L., Pohl, Martin, Gorzelanny, Christian, Tschongov, Todor A., Häffner, Karsten

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185 CPV-104, an improved recombinant variant of human complement factor H produced in moss, is a prime candidate for clinical application in complement mediated diseases by Tschongov, Todor, Konwar, Swagata, Kleindienst, Jessika, Dabrowska-Schlepp, Paulina, Busch, Andreas, Häffner, Karsten

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CPV-104, a Recombinant Variant of Human Complement Factor H Produced in Moss, to Be Studied in a Phase 1/2 Clinical Trial in Patients with C3 Glomerulopathy (C3G): SA-PO918 by Heidenreich, Karin, Cochlovius, Björn, Bauer, Martin, Busch, Andreas, Dabrowska-Schlepp, Paulina, Schaaf, Andreas, Zeitter, Fabienne C., Häffner, Karsten, Koehl, Joerg, Schmidt, Tilman, Wiech, Thorsten, Zipfel, Peter F.

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