Die Rolle des R2TP-Proteinkomplexes bei der Pathogenese zystischer Nierenerkrankungen und in der Biologie primärer Zilien

• Antragsteller: Professor Dr. Bernhard Schermer
• Fachliche Zuordnung: Nephrologie
• Förderung: Förderung von 2016 bis 2021
• Projektkennung: Deutsche Forschungsgemeinschaft (DFG) - Projektnummer 314732659

• Erstellungsjahr: 2020

Keine Zusammenfassung vorhanden

Projektbezogene Publikationen (Auswahl)

• Challenges in establishing genotype-phenotype correlations in ARPKD: case report on a toddler with two severe PKHD1 mutations. Pediatr. Nephrol. 32, 1269-1273 (2017)
  Ebner, Kathrin; Dafinger, Claudia; Ortiz-Bruechle, Nadina; Koerber, Friederike; Schermer, Bernhard; Benzing, Thomas; Dötsch, Jörg; Zerres, Klaus; Weber, Lutz Thorsten; Beck, Bodo B. & Liebau, Max Christoph
  
• Magnetic resonance T2 mapping and diffusionweighted imaging for early detection of cystogenesis and response to therapy in a mouse model of polycystic kidney disease. Kidney Int. 92, 1544-1554 (2017)
  Franke, Mareike; Baeßler, Bettina; Vechtel, Jan; Dafinger, Claudia; Höhne, Martin; Borgal, Lori; Göbel, Heike; Koerber, Friederike; Maintz, David; Benzing, Thomas; Schermer, Bernhard & Persigehl, Thorsten
  
• Proline-dependent and basophilic kinases phosphorylate human TRPC6 at serine 14 to control channel activity through increased membrane expression. FASEB J. 32, 208-219 (2018)
  Hagmann, Henning; Mangold, Nicole; Rinschen, Markus M.; Koenig, Tim; Kunzelmann, Karl; Schermer, Bernhard; Benzing, Thomas & Brinkkoetter, Paul T.
  
• The ciliary membrane-associated proteome reveals actin-binding proteins as key components of cilia. EMBO Rep 18, 1521-1535 (2017)
  Kohli, Priyanka; Höhne, Martin; Jüngst, Christian; Bertsch, Sabine; Ebert, Lena K; Schauss, Astrid C; Benzing, Thomas; Rinschen, Markus M & Schermer, Bernhard
  
• An optimized electroporation approach for efficient CRISPR/Cas9 genome editing in murine zygotes. PLoS One 13, e0196891 (2018)
  Tröder, Simon E.; Ebert, Lena K.; Butt, Linus; Assenmacher, Sonja; Schermer, Bernhard & Zevnik, Branko
  
• Targeted deletion of the AAA-ATPase Ruvbl1 in mice disrupts ciliary integrity and causes renal disease and hydrocephalus. Exp. Mol. Med. 50, 1-17 (2018)
  Dafinger, Claudia; Rinschen, Markus M.; Borgal, Lori; Ehrenberg, Carolin; Basten, Sander G.; Franke, Mareike; Höhne, Martin; Rauh, Manfred; Göbel, Heike; Bloch, Wilhelm; Wunderlich, F. Thomas; Peters, Dorien J. M.; Tasche, Dirk; Mishra, Tripti; Habbig, Sandra; Dötsch, Jörg; Müller, Roman-Ulrich; Brüning, Jens C.; Persigehl, Thorsten; ... & Liebau, Max C.
  
• A protein- RNA interaction atlas of the ribosome biogenesis factor AATF. Sci. Rep. 9, 11071 (2019)
  Kaiser, Rainer W. J.; Ignarski, Michael; Van Nostrand, Eric L.; Frese, Christian K.; Jain, Manaswita; Cukoski, Sadrija; Heinen, Heide; Schaechter, Melanie; Seufert, Lisa; Bunte, Konstantin; Frommolt, Peter; Keller, Patrick; Helm, Mark; Bohl, Katrin; Höhne, Martin; Schermer, Bernhard; Benzing, Thomas; Höpker, Katja; Dieterich, Christoph; ... & Fabretti, Francesca
  
• Activation of Hypoxia-Inducible Factor Signaling Modulates the RNA Protein Interactome in Caenorhabditis elegans. iScience 22, 466-476 (2019)
  Esmaillie, Reza; Ignarski, Michael; Bohl, Katrin; Krüger, Tim; Ahmad, Daniyal; Seufert, Lisa; Schermer, Bernhard; Benzing, Thomas; Müller, Roman-Ulrich & Fabretti, Francesca
  
• Antagonizing Transcription Factor in tubular epithelial cells induces accumulation of DNA damage and nephronophthisis. Kidney Int. 95, 846-858 (2019)
  Jain, Manaswita; Kaiser, Rainer W.J.; Bohl, Katrin; Hoehne, Martin; Göbel, Heike; Bartram, Malte P.; Habbig, Sandra; Müller, Roman-Ulrich; Fogo, Agnes B.; Benzing, Thomas; Schermer, Bernhard; Höpker, Katja & Slaats, Gisela G.
  
• Enzyme Replacement Therapy Clears Gb3 Deposits from a Podocyte Cell Culture Model of Fabry Disease but Fails to Restore Altered Cellular Signaling. Cell. Physiol. Biochem. 52, 1139-1150 (2019)
  Braun, F., Blomberg, L., Brodesser, S., Liebau, M.C., Schermer, B., Benzing, T. & Kurschat, C.E.
  
• Hippo signaling-a central player in cystic kidney disease? Pediatr. Nephrol. 35, 1143-1152 (2020)
  Müller, Roman-Ulrich & Schermer, Bernhard
  
• The RNA-Protein Interactome of Differentiated Kidney Tubular Epithelial Cells. J. Am. Soc. Nephrol. 30, 564-576 (2019)
  Ignarski, Michael; Rill, Constantin; Kaiser, Rainer W.J.; Kaldirim, Madlen; Neuhaus, René; Esmaillie, Reza; Li, Xinping; Klein, Corinna; Bohl, Katrin; Petersen, Maike; Frese, Christian K.; Höhne, Martin; Atanassov, Ilian; Rinschen, Markus M.; Höpker, Katja; Schermer, Bernhard; Benzing, Thomas; Dieterich, Christoph; Fabretti, Francesca; ... & Müller, Roman-Ulrich
  
• The Integrated RNA Landscape of Renal Preconditioning against Ischemia-Reperfusion Injury. J. Am. Soc. Nephrol. 31, 716-730 (2020)
  Johnsen, Marc; Kubacki, Torsten; Yeroslaviz, Assa; Späth, Martin Richard; Mörsdorf, Jannis; Göbel, Heike; Bohl, Katrin; Ignarski, Michael; Meharg, Caroline; Habermann, Bianca; Altmüller, Janine; Beyer, Andreas; Benzing, Thomas; Schermer, Bernhard; Burst, Volker & Müller, Roman-Ulrich