Thomas Benzing

Summary

Affiliation: University of Freiburg
Country: Germany

Publications

  1. ncbi request reprint Signaling at the slit diaphragm
    Thomas Benzing
    Renal Division, University Hospital Freiburg, Hugstetterstrassse 55, 79106 Freiburg, Germany
    J Am Soc Nephrol 15:1382-91. 2004
  2. pmc Neph-Nephrin proteins bind the Par3-Par6-atypical protein kinase C (aPKC) complex to regulate podocyte cell polarity
    Björn Hartleben
    Renal Division, University Hospital Freiburg, D 79106 Freiburg, Germany
    J Biol Chem 283:23033-8. 2008
  3. pmc The von Hippel-Lindau tumor suppressor protein controls ciliogenesis by orienting microtubule growth
    Bernhard Schermer
    Renal Division and 2Children s Hospital, University Hospital Freiburg, 79106 Freiburg, Germany
    J Cell Biol 175:547-54. 2006
  4. pmc Phosphorylation by casein kinase 2 induces PACS-1 binding of nephrocystin and targeting to cilia
    Bernhard Schermer
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    EMBO J 24:4415-24. 2005
  5. ncbi request reprint Nephrocystin specifically localizes to the transition zone of renal and respiratory cilia and photoreceptor connecting cilia
    Manfred Fliegauf
    Department of Pediatrics and Adolescent Medicine, Mathildenstrasse 1, 79106 Freiburg, Germany
    J Am Soc Nephrol 17:2424-33. 2006
  6. pmc Nephrocystin-4 regulates Pyk2-induced tyrosine phosphorylation of nephrocystin-1 to control targeting to monocilia
    Max C Liebau
    Renal Division, Department of Medicine and Center for Molecular Medicine, University of Cologne, 50937 Cologne, Germany
    J Biol Chem 286:14237-45. 2011
  7. pmc NPHP4, a cilia-associated protein, negatively regulates the Hippo pathway
    Sandra Habbig
    Renal Division, Department of Medicine, University of Cologne, 50937 Cologne, Germany
    J Cell Biol 193:633-42. 2011
  8. ncbi request reprint Homodimerization and heterodimerization of the glomerular podocyte proteins nephrin and NEPH1
    Peter Gerke
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    J Am Soc Nephrol 14:918-26. 2003
  9. doi request reprint Caenorhabditis elegans, a model organism for kidney research: from cilia to mechanosensation and longevity
    Roman Ulrich Muller
    Renal Division, Department of Medicine and Centre for Molecular Medicine, Cologne Excellence Cluster on Cellular Stress Responses in Aging Associated Diseases, University of Cologne, Cologne, Germany
    Curr Opin Nephrol Hypertens 20:400-8. 2011
  10. ncbi request reprint Molecular basis of the functional podocin-nephrin complex: mutations in the NPHS2 gene disrupt nephrin targeting to lipid raft microdomains
    Tobias B Huber
    Renal Division, University Hospital Freiburg, Germany
    Hum Mol Genet 12:3397-405. 2003

Collaborators

Detail Information

Publications36

  1. ncbi request reprint Signaling at the slit diaphragm
    Thomas Benzing
    Renal Division, University Hospital Freiburg, Hugstetterstrassse 55, 79106 Freiburg, Germany
    J Am Soc Nephrol 15:1382-91. 2004
  2. pmc Neph-Nephrin proteins bind the Par3-Par6-atypical protein kinase C (aPKC) complex to regulate podocyte cell polarity
    Björn Hartleben
    Renal Division, University Hospital Freiburg, D 79106 Freiburg, Germany
    J Biol Chem 283:23033-8. 2008
    ....
  3. pmc The von Hippel-Lindau tumor suppressor protein controls ciliogenesis by orienting microtubule growth
    Bernhard Schermer
    Renal Division and 2Children s Hospital, University Hospital Freiburg, 79106 Freiburg, Germany
    J Cell Biol 175:547-54. 2006
    ..Furthermore, pVHL interacts with the Par3-Par6-atypical PKC complex, suggesting a mechanism for linking polarity pathways to microtubule capture and ciliogenesis...
  4. pmc Phosphorylation by casein kinase 2 induces PACS-1 binding of nephrocystin and targeting to cilia
    Bernhard Schermer
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    EMBO J 24:4415-24. 2005
    ..Inhibition of CK2 activity abrogates this interaction and results in the loss of correct nephrocystin targeting. These data suggest that CK2-dependent transport processes represent a novel pathway of targeting proteins to the cilia...
  5. ncbi request reprint Nephrocystin specifically localizes to the transition zone of renal and respiratory cilia and photoreceptor connecting cilia
    Manfred Fliegauf
    Department of Pediatrics and Adolescent Medicine, Mathildenstrasse 1, 79106 Freiburg, Germany
    J Am Soc Nephrol 17:2424-33. 2006
    ..In conclusion, nephrocystin deficiency or dysfunction at the transition zone of renal monocilia and the photoreceptor connecting cilium might explain renal failure and retinal degeneration that are observed in patients with NPHP1...
  6. pmc Nephrocystin-4 regulates Pyk2-induced tyrosine phosphorylation of nephrocystin-1 to control targeting to monocilia
    Max C Liebau
    Renal Division, Department of Medicine and Center for Molecular Medicine, University of Cologne, 50937 Cologne, Germany
    J Biol Chem 286:14237-45. 2011
    ..These data strongly suggest that NPHP4 acts upstream of NPHP1 in a common pathway and support the concept of a role for nephrocystin proteins in intracellular vesicular transport...
  7. pmc NPHP4, a cilia-associated protein, negatively regulates the Hippo pathway
    Sandra Habbig
    Renal Division, Department of Medicine, University of Cologne, 50937 Cologne, Germany
    J Cell Biol 193:633-42. 2011
    ..These data identify NPHP4 as a negative regulator of the Hippo pathway and suggest that NPHP4 regulates cell proliferation through its effects on Hippo signaling...
  8. ncbi request reprint Homodimerization and heterodimerization of the glomerular podocyte proteins nephrin and NEPH1
    Peter Gerke
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    J Am Soc Nephrol 14:918-26. 2003
    ....
  9. doi request reprint Caenorhabditis elegans, a model organism for kidney research: from cilia to mechanosensation and longevity
    Roman Ulrich Muller
    Renal Division, Department of Medicine and Centre for Molecular Medicine, Cologne Excellence Cluster on Cellular Stress Responses in Aging Associated Diseases, University of Cologne, Cologne, Germany
    Curr Opin Nephrol Hypertens 20:400-8. 2011
    ..Here, we review the role of the nematode in renal research and discuss future perspectives for its use in molecular nephrology...
  10. ncbi request reprint Molecular basis of the functional podocin-nephrin complex: mutations in the NPHS2 gene disrupt nephrin targeting to lipid raft microdomains
    Tobias B Huber
    Renal Division, University Hospital Freiburg, Germany
    Hum Mol Genet 12:3397-405. 2003
    ..Our findings demonstrate that the failure of mutant podocin to recruit nephrin into lipid rafts may be essential for the pathogenesis of NPHS2...
  11. pmc The BAR domain protein PICK1 regulates cell recognition and morphogenesis by interacting with Neph proteins
    Martin Höhne
    Renal Division, Department of Medicine and Center for Molecular Medicine, University of Cologne, 50937 Cologne, Germany
    Mol Cell Biol 31:3241-51. 2011
    ..These data indicate that Neph proteins and PICK1 synergistically regulate cell recognition and contact formation...
  12. pmc Podocin and MEC-2 bind cholesterol to regulate the activity of associated ion channels
    Tobias B Huber
    Renal Division, University Hospital Freiburg, 79106 Freiburg, Germany
    Proc Natl Acad Sci U S A 103:17079-86. 2006
    ..Thus, MEC-2, Podocin, and probably many other PHB-domain proteins by binding to themselves, cholesterol, and target proteins regulate the formation and function of large protein-cholesterol supercomplexes in the plasma membrane...
  13. pmc Nephrin and CD2AP associate with phosphoinositide 3-OH kinase and stimulate AKT-dependent signaling
    Tobias B Huber
    Renal Division Molecular Medicine, University Hospital Freiburg, D 79106 Freiburg, Germany
    Mol Cell Biol 23:4917-28. 2003
    ....
  14. ncbi request reprint NEPH2 is located at the glomerular slit diaphragm, interacts with nephrin and is cleaved from podocytes by metalloproteinases
    Peter Gerke
    Renal Division, University Hospital Freiburg, Hugstetter Str 55, 79106 Freiburg, Germany
    J Am Soc Nephrol 16:1693-702. 2005
    ..The results suggest a role for NEPH2 in the organization and/or maintenance of the glomerular slit diaphragm that may differ from the functions of NEPH1 and nephrin...
  15. ncbi request reprint Neuronal expression and interaction with the synaptic protein CASK suggest a role for Neph1 and Neph2 in synaptogenesis
    Peter Gerke
    Renal Division, University of Freiburg, D 79104 Freiburg, Germany
    J Comp Neurol 498:466-75. 2006
    ..Our results show that Neph proteins are expressed in the developing nervous system of mammals and suggest that these proteins may have a conserved function in synapse formation or neurogenesis...
  16. pmc Trafficking of TRPP2 by PACS proteins represents a novel mechanism of ion channel regulation
    Michael Köttgen
    Renal Division, University Hospital of Freiburg, Freiburg, Germany
    EMBO J 24:705-16. 2005
    ..Furthermore, PACS proteins may represent a novel molecular mechanism for ion channel trafficking, directing acidic cluster-containing ion channels to distinct subcellular compartments...
  17. pmc The von Hippel Lindau tumor suppressor limits longevity
    Roman Ulrich Muller
    Renal Division, Department of Medicine, University of Cologne, Kerpener Strasse 62, Cologne, Germany
    J Am Soc Nephrol 20:2513-7. 2009
    ..These findings define VHL-1 as a player in longevity signaling and connect aging, regulation of lifespan, and stress responses with formation of renal cell carcinomas...
  18. ncbi request reprint Podocin organizes ion channel-lipid supercomplexes: implications for mechanosensation at the slit diaphragm
    Tobias B Huber
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    Nephron Exp Nephrol 106:e27-31. 2007
    ..This review will summarize the most recent findings on protein-lipid supercomplexes at the slit diaphragm and discuss a potential novel function of the slit diaphragm as a mechanosensor...
  19. pmc Inversin, the gene product mutated in nephronophthisis type II, functions as a molecular switch between Wnt signaling pathways
    Matias Simons
    Renal Division, University Hospital Freiburg, Hugstetter Strasse 55, 79106 Freiburg, Germany
    Nat Genet 37:537-43. 2005
    ..Fluid flow increases inversin levels in ciliated tubular epithelial cells and seems to regulate this crucial switch between Wnt signaling pathways during renal development...
  20. ncbi request reprint Wnt signaling in polycystic kidney disease
    Thomas Benzing
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    J Am Soc Nephrol 18:1389-98. 2007
    ..These spatial cues may be needed to position nascent tubules with a defined geometry...
  21. ncbi request reprint When cilia go bad: cilia defects and ciliopathies
    Manfred Fliegauf
    Department of Paediatrics and Adolescent Medicine, University Hospital Freiburg, 79106 Freiburg, Germany
    Nat Rev Mol Cell Biol 8:880-93. 2007
    ..Several molecular mechanisms involved in cilia-related disorders have been identified that affect the structure and function of distinct cilia types...
  22. ncbi request reprint Von hippel-lindau: a tumor suppressor links microtubules to ciliogenesis and cancer development
    E Wolfgang Kuehn
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    Cancer Res 67:4537-40. 2007
    ..These results add to a growing body of evidence linking cilia and the cell cycle and suggest that the tumor suppressor role of pVHL may involve previously unrecognized pathways...
  23. ncbi request reprint Cilium-generated signaling: a cellular GPS?
    Thomas Benzing
    Renal Division, University Hospital Freiburg, Freiburg, Germany
    Curr Opin Nephrol Hypertens 15:245-9. 2006
    ..This review focuses on the most recent advance in understanding a role for signaling through cilia for kidney biology...
  24. ncbi request reprint Mutations in a novel gene, NPHP3, cause adolescent nephronophthisis, tapeto-retinal degeneration and hepatic fibrosis
    Heike Olbrich
    Department of Pediatrics and Adolescent Medicine, University Hospital Freiburg, 79106 Freiburg, Germany
    Nat Genet 34:455-9. 2003
    ..Interventional studies in the pcy mouse have shown beneficial effects by modification of protein intake and administration of methylprednisolone, suggesting therapeutic strategies for treating individuals with NPHP3...
  25. doi request reprint Rituximab treatment for adults with refractory nephrotic syndrome: a single-center experience and review of the literature
    Tuelay Kisner
    Renal Division, Department of Medicine and Center for Molecular Medicine, University of Cologne, Cologne, Germany
    Nephron Clin Pract 120:c79-85. 2012
    ..Recently, B cell-directed therapy using the anti-CD20 antibody rituximab has been suggested as induction regimen in pediatric FSGS and MCD patients. Data on rituximab use in adults are still limited...
  26. ncbi request reprint Dyslipidemia treatment and cardiovascular disease in the renal patient
    Volker Burst
    Renal Division, Department of Medicine, University of Cologne, Germany
    Curr Pharm Des 17:894-907. 2011
    ....
  27. ncbi request reprint The carboxyl terminus of Neph family members binds to the PDZ domain protein zonula occludens-1
    Tobias B Huber
    Renal Division, University Hospital Freiburg, Hugstetter Str 55, 79106 Freiburg, Germany
    J Biol Chem 278:13417-21. 2003
    ..Thus, our data suggest that ZO-1 may organize Neph proteins and recruit signal transduction components to the slit diaphragm of podocytes...
  28. doi request reprint The promise of angiogenic markers for the early diagnosis and prediction of preeclampsia
    Henning Hagmann
    Renal Division, Department of Medicine and Centre for Molecular Medicine, University of Cologne, Cologne, Germany
    Clin Chem 58:837-45. 2012
    ..An imbalance in circulating factors that regulate blood vessel formation and health, referred to as angiogenic factors, plays a central role in the pathogenesis of preeclampsia...
  29. ncbi request reprint Genetic testing comes of age: WT1 mutations in steroid-resistant nephrotic syndrome: commentary on the article by Mucha et al. on page 325
    Thomas Benzing
    Department of Medicine, University Hospital Freiburg, Germany
    Pediatr Res 59:165-6. 2006
  30. pmc KIBRA modulates directional migration of podocytes
    Kerstin Duning
    Medizinische Klinik und Poliklinik D, Universitatsklinikum Munster, Munster, Germany
    J Am Soc Nephrol 19:1891-903. 2008
    ..Stable knockdown of KIBRA in immortalized podocytes impaired directed cell migration, suggesting that KIBRA modulates the motility of podocytes by linking polarity proteins and cytoskeleton-associated protein complexes...
  31. ncbi request reprint Stra13, a prostaglandin E2-induced gene, regulates the cellular redox state of podocytes
    Martin J Bek
    Department of Medicine, Division of Nephrology, Freiburg University Hospital, Freiburg, Germany
    FASEB J 17:682-4. 2003
    ..ROS are involved in the pathogenesis of glomerular inflammation in several forms of glomerulonephritis. Therefore, knowledge about protective mechanisms may provide insight into new therapeutic strategies for glomerulopathies...
  32. pmc Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination
    Edgar A Otto
    Department of Pediatrics, 8220C MSRB III, 1150 West Medical Center Drive, University of Michigan, Ann Arbor, Michigan 48109, USA
    Nat Genet 34:413-20. 2003
    ..The interaction and colocalization in cilia of inversin, nephrocystin and beta-tubulin connect pathogenetic aspects of NPHP to PKD, to primary cilia function and to left-right axis determination...
  33. ncbi request reprint The slit diaphragm: a signaling platform to regulate podocyte function
    Tobias B Huber
    Pathology and Immunology, Washington University St Louis, St Louis, Missouri, USA
    Curr Opin Nephrol Hypertens 14:211-6. 2005
    ..This review focuses on the most recent evidence elucidating the dynamic functions of the slit diaphragm and stresses the importance of slit diaphragm proteins in mediating signal transduction in the podocyte...
  34. pmc Loss of nephrocystin-3 function can cause embryonic lethality, Meckel-Gruber-like syndrome, situs inversus, and renal-hepatic-pancreatic dysplasia
    Carsten Bergmann
    Department of Human Genetics, RWTH Aachen University, 52074 Aachen, Germany
    Am J Hum Genet 82:959-70. 2008
    ....
  35. pmc Nephrin mutations can cause childhood-onset steroid-resistant nephrotic syndrome
    Aurélie Philippe
    Inserm U574, Hopital Necker Enfants Malades, Paris, France
    J Am Soc Nephrol 19:1871-8. 2008
    ..The presence of at least one "mild" mutation in these patients likely explains the later onset and milder course of disease. These results broaden the spectrum of renal disease related to nephrin mutations...
  36. pmc The polycystin-1 C-terminal fragment triggers branching morphogenesis and migration of tubular kidney epithelial cells
    Christian Nickel
    Section of Nephrology, Department of Medicine, Yale University, New Haven, Connecticut 06529 8029, USA
    J Clin Invest 109:481-9. 2002
    ..Our findings demonstrate that the C-terminal domain of polycystin-1, acting in a ligand-independent fashion, triggers unique signaling pathways for morphogenesis, and likely plays a central role in polycystin-1 function...