Thomas J Jentsch

Summary

Affiliation: University of Hamburg
Country: Germany

Publications

  1. ncbi request reprint Chloride transport in the kidney: lessons from human disease and knockout mice
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    J Am Soc Nephrol 16:1549-61. 2005
  2. ncbi request reprint Physiological functions of CLC Cl- channels gleaned from human genetic disease and mouse models
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie Hamburg ZMNH, Universitat Hamburg, Falkenried 94, D 20251 Hamburg, Germany
    Annu Rev Physiol 67:779-807. 2005
  3. ncbi request reprint CLC chloride channels and transporters
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie, ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg
    Curr Opin Neurobiol 15:319-25. 2005
  4. ncbi request reprint Ion channels: function unravelled by dysfunction
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Nat Cell Biol 6:1039-47. 2004
  5. pmc Chloride channel diseases resulting from impaired transepithelial transport or vesicular function
    Thomas J Jentsch
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Hamburg, Germany
    J Clin Invest 115:2039-46. 2005
  6. ncbi request reprint Molecular structure and physiological function of chloride channels
    Thomas J Jentsch
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Hamburg, Germany
    Physiol Rev 82:503-68. 2002
  7. pmc Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6
    Mallorie Poet
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Proc Natl Acad Sci U S A 103:13854-9. 2006
  8. ncbi request reprint Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins
    Olaf Scheel
    Zentrum für Molekulare Neurobiologie, ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Nature 436:424-7. 2005
  9. pmc Functional and structural conservation of CBS domains from CLC chloride channels
    Raúl Estévez
    Zentrum für Molekulare Neurobiologie Hamburg ZMNH, Hamburg University, Falkenried 94, D 20246 Hamburg, Germany
    J Physiol 557:363-78. 2004
  10. ncbi request reprint Neurogenic mechanisms contribute to hypertension in mice with disruption of the K-Cl cotransporter KCC3
    Marco B Rust
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Universitatsklinikum Hamburg Eppendorf, Hamburg, Germany
    Circ Res 98:549-56. 2006

Collaborators

Detail Information

Publications35

  1. ncbi request reprint Chloride transport in the kidney: lessons from human disease and knockout mice
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    J Am Soc Nephrol 16:1549-61. 2005
    ..Finally, both KCC3 and KCC4 are crucial for proximal tubular cell volume regulation...
  2. ncbi request reprint Physiological functions of CLC Cl- channels gleaned from human genetic disease and mouse models
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie Hamburg ZMNH, Universitat Hamburg, Falkenried 94, D 20251 Hamburg, Germany
    Annu Rev Physiol 67:779-807. 2005
    ....
  3. ncbi request reprint CLC chloride channels and transporters
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie, ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg
    Curr Opin Neurobiol 15:319-25. 2005
    ..Surprisingly, the CLC from Escherichia coli functions as a Cl-/H+ exchanger, thus demonstrating the thin line separating transporters and channels...
  4. ncbi request reprint Ion channels: function unravelled by dysfunction
    Thomas J Jentsch
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Nat Cell Biol 6:1039-47. 2004
    ....
  5. pmc Chloride channel diseases resulting from impaired transepithelial transport or vesicular function
    Thomas J Jentsch
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Hamburg, Germany
    J Clin Invest 115:2039-46. 2005
    ..This review will focus on diseases related to transepithelial transport and on disorders involving vesicular Cl- channels...
  6. ncbi request reprint Molecular structure and physiological function of chloride channels
    Thomas J Jentsch
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Hamburg, Germany
    Physiol Rev 82:503-68. 2002
    ..Other gene families (CLIC or CLCA) were also reported to encode Cl- channels but are less well characterized. This review focuses on molecularly identified Cl- channels and their physiological roles...
  7. pmc Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6
    Mallorie Poet
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Proc Natl Acad Sci U S A 103:13854-9. 2006
    ..CLCN6 is a candidate gene for mild forms of human NCL. Analysis of 75 NCL patients identified ClC-6 amino acid exchanges in two patients but failed to prove a causative role of CLCN6 in that disease...
  8. ncbi request reprint Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins
    Olaf Scheel
    Zentrum für Molekulare Neurobiologie, ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Nature 436:424-7. 2005
    ..ClC-4 and ClC-5 may still compensate the charge accumulation by endosomal proton pumps, but are expected to couple directly vesicular pH gradients to Cl- gradients...
  9. pmc Functional and structural conservation of CBS domains from CLC chloride channels
    Raúl Estévez
    Zentrum für Molekulare Neurobiologie Hamburg ZMNH, Hamburg University, Falkenried 94, D 20246 Hamburg, Germany
    J Physiol 557:363-78. 2004
    ..Thus, we propose that the structure of CBS domains from CLC channels is highly conserved and that they play a functional role in the common gate...
  10. ncbi request reprint Neurogenic mechanisms contribute to hypertension in mice with disruption of the K-Cl cotransporter KCC3
    Marco B Rust
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Universitatsklinikum Hamburg Eppendorf, Hamburg, Germany
    Circ Res 98:549-56. 2006
    ..Our data indicate that local control of myogenic tone does not require KCC3 and that hypertension in Kcc3(-/-) mice depends on an elevated sympathetic tone...
  11. pmc Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration
    Dagmar Kasper
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, Hamburg, Germany
    EMBO J 24:1079-91. 2005
    ..There are important medical implications as defects in the H(+)-ATPase and ClC-7 can underlie human osteopetrosis...
  12. pmc Disruption of erythroid K-Cl cotransporters alters erythrocyte volume and partially rescues erythrocyte dehydration in SAD mice
    Marco B Rust
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Hamburg, Germany
    J Clin Invest 117:1708-17. 2007
    ..Although disruption of K-Cl cotransport rescued the dehydration phenotype of most SAD rbc, the proportion of the densest red blood cell population remained unaffected...
  13. ncbi request reprint Leukoencephalopathy upon disruption of the chloride channel ClC-2
    Judith Blanz
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, D 20252 Hamburg, Germany
    J Neurosci 27:6581-9. 2007
    ..Sequencing of a large collection of human DNA and electrophysiological analysis showed that several ClC-2 sequence abnormalities previously found in patients with epilepsy most likely represent innocuous polymorphisms...
  14. doi request reprint Role of the vesicular chloride transporter ClC-3 in neuroendocrine tissue
    Tanja Maritzen
    Zentrum für Molekulare Neurobiologie ZMNH, Universitat Hamburg, D 20246 Hamburg, Germany
    J Neurosci 28:10587-98. 2008
    ..This observation points to an indirect influence of ClC-3 on LDCV exocytosis in chromaffin cells, possibly by affecting an intracellular trafficking step...
  15. ncbi request reprint ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function
    Philipp F Lange
    Zentrum fur Molekulare Neurobiologie Hamburg, ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Nature 440:220-3. 2006
    ..3). The finding that grey-lethal mice, just like ClC-7-deficient mice, show lysosomal storage and neurodegeneration in addition to osteopetrosis implies a more general importance for ClC-7-Ostm1 complexes...
  16. pmc Loss of K-Cl co-transporter KCC3 causes deafness, neurodegeneration and reduced seizure threshold
    Thomas Boettger
    Zentrum für Molekulare Neurobiologie, ZMNH, Universitat Hamburg, Falkenried 94, D 20251 Hamburg, Germany
    EMBO J 22:5422-34. 2003
    ..These cells slowly degenerated, as did sensory hair cells. The present mouse model has revealed important cellular and systemic functions of KCC3 and is highly relevant for Anderman syndrome...
  17. ncbi request reprint Conservation of chloride channel structure revealed by an inhibitor binding site in ClC-1
    Raúl Estévez
    Zentrum fur Molekulare Neurobiologie Hamburg, ZMNH, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Neuron 38:47-59. 2003
    ..Mutations in presumably Cl--coordinating residues yield additional insights into the structure and function of ClC-1. Our work shows that the structure of bacterial CLCs can be extrapolated with fidelity to mammalian Cl- channels...
  18. pmc Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness
    Tatjana Kharkovets
    Zentrum für Molekulare Neurobiologie, ZMNH, Universitat Hamburg, Hamburg, Germany
    EMBO J 25:642-52. 2006
    ..These cells were only slightly depolarized and showed near-normal presynaptic function. We conclude that the hearing loss in DFNA2 is predominantly caused by a slow degeneration of OHCs resulting from chronic depolarization...
  19. ncbi request reprint Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models
    Anselm A Zdebik
    Zentrum für Molekulare Neurobiologie ZMNH, Falkenried 94, D 20246 Hamburg, Germany
    J Biol Chem 279:22276-83. 2004
    ..It is concluded that ClC-2 is unlikely to be a candidate rescue channel in cystic fibrosis. Our data are consistent with a model in which ClC-2 is located in the basolateral membrane...
  20. ncbi request reprint Expression of the KCl cotransporter KCC2 parallels neuronal maturation and the emergence of low intracellular chloride
    Valentin Stein
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, D 20246 Hamburg, Germany
    J Comp Neurol 468:57-64. 2004
    ..Phosphorylated KCC2 protein was already present early in development when the functional GABA switch had not yet occurred. Thus, tyrosine-phosphorylation seems to be less important than the transcriptional upregulation of KCC2...
  21. ncbi request reprint CLC chloride channels: correlating structure with function
    Raúl Estévez
    Zentrum für Molekulare Neurobiologie, Universitat Hamburg, Falkenried 94, Germany
    Curr Opin Struct Biol 12:531-9. 2002
    ..A glutamate residue that protrudes into the pore is proposed to participate in gating. The structure confirms several previous conclusions from mutagenesis studies and provides an excellent framework for their interpretation...
  22. pmc A carboxy-terminal domain determines the subunit specificity of KCNQ K+ channel assembly
    Michael Schwake
    Centre for Molecular Neurobiology Hamburg, ZMNH, Falkenried 94, Germany
    EMBO Rep 4:76-81. 2003
    ..Conversely, a KCNQ3-sid(Q1) chimaera no longer affects KCNQ2 but interacts with KCNQ1. We conclude that the si domain suffices to determine the subunit specificity of KCNQ channel assembly...
  23. ncbi request reprint The ClC-5 chloride channel knock-out mouse - an animal model for Dent's disease
    Willy Günther
    Zentrum fur Molekulare Neurobiologie Hamburg, ZMNH, Universitat Hamburg, Falkenried 94, 20246 Hamburg, Germany
    Pflugers Arch 445:456-62. 2003
    ..We discuss how the primary defect in endocytosis leads via secondary changes in calciotropic hormones to the tertiary symptoms hyperphosphaturia, hypercalciuria and kidney stones...
  24. ncbi request reprint Ion channel diseases
    Christian A Hübner
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitat Hamburg, Falkenried 94, D 20246 Hamburg, Germany
    Hum Mol Genet 11:2435-45. 2002
    ..This review gives a short overview of known channelopathies, and focuses in particular on recent findings and on channelopathies that have significantly advanced our physiological insight...
  25. ncbi request reprint Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4
    Thomas Boettger
    Zentrum für Molekulare Neurobiologie, ZMNH, Universitat Hamburg, Falkenried 94, 20246 Hamburg, Germany
    Nature 416:874-8. 2002
    ..It probably results from an impairment of Cl(-) recycling across the basolateral membrane of acid-secreting alpha-intercalated cells of the distal nephron...
  26. ncbi request reprint Determinants of anion-proton coupling in mammalian endosomal CLC proteins
    Anselm A Zdebik
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, D 13125 Berlin, Germany
    J Biol Chem 283:4219-27. 2008
    ....
  27. pmc Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV
    Gesa Rickheit
    Leibniz Institut fur Molekulare Pharmakologie, Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    EMBO J 27:2907-17. 2008
    ..Bsnd(-/-) mice thus demonstrate a novel function of Cl(-) channels in generating the endocochlear potential and reveal the mechanism leading to deafness in human Bartter syndrome IV...
  28. pmc Chloride and the endosomal-lysosomal pathway: emerging roles of CLC chloride transporters
    Thomas J Jentsch
    FMP MDC, Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Zentrum für Molekulare Medizin, Robert Rossle Strasse 10, D 13125 Berlin, FRG
    J Physiol 578:633-40. 2007
    ..Surprisingly, ClC-4 and ClC-5 (and probably ClC-3) do not function as Cl- channels, but rather as electrogenic Cl--H+ exchangers. This hints at an important role for luminal chloride in the endosomal-lysosomal system...
  29. ncbi request reprint Structural determinants of M-type KCNQ (Kv7) K+ channel assembly
    Michael Schwake
    Institut fur Biochemie, Christian Albrechts Universitat zu Kiel, D 24098 Kiel, Germany
    J Neurosci 26:3757-66. 2006
    ....
  30. ncbi request reprint Chloride channels are different
    Thomas J Jentsch
    Nature 415:276-7. 2002
  31. ncbi request reprint Unique structure and function of chloride transporting CLC proteins
    Michael Pusch
    Institute of Biophysics, Italian Research Council, Genoa I 16149, Italy
    IEEE Trans Nanobioscience 4:49-57. 2005
    ..These recent breakthroughs will allow us to study in further detail the structure, function, and the physiological and pathophysiological role of CLC proteins...
  32. pmc Mice with a targeted disruption of the Cl-/HCO3- exchanger AE3 display a reduced seizure threshold
    Moritz Hentschke
    Department of Human Genetics, UKE Hamburg, Butenfeld 42, 22529 Hamburg, Germany
    Mol Cell Biol 26:182-91. 2006
    ..These findings strongly support the hypothesis that AE3 modulates seizure susceptibility and, therefore, are of significance for understanding the role of intracellular pH in epilepsy...
  33. ncbi request reprint Molecular determinants of KCNQ (Kv7) K+ channel sensitivity to the anticonvulsant retigabine
    Anne Schenzer
    Institute of Biochemistry, Christian Albrechts University Kiel, D 24098 Kiel, Germany
    J Neurosci 25:5051-60. 2005
    ..Transfer of the tryptophan into the KCNQ1 scaffold resulted in retigabine-sensitive heteromers, suggesting that the tryptophan is necessary in all KCNQ subunits forming a functional tetramer to confer drug sensitivity...
  34. ncbi request reprint CLC chloride channels and transporters: from genes to protein structure, pathology and physiology
    Thomas J Jentsch
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    Crit Rev Biochem Mol Biol 43:3-36. 2008
    ....
  35. ncbi request reprint Plasmodium induces swelling-activated ClC-2 anion channels in the host erythrocyte
    Stephan M Huber
    Departments of Physiology and Parasitology, Institute of Tropical Medicine, University of Tubingen, 72076 Germany
    J Biol Chem 279:41444-52. 2004
    ..In conclusion, activation of host ClC-2 channels participates in the altered permeability of Plasmodium-infected erythrocytes but is not required for intraerythrocytic parasite survival...