Thomas J Jentsch

Summary

Country: Germany

Publications

  1. pmc A missense mutation accelerating the gating of the lysosomal Cl-/H+-exchanger ClC-7/Ostm1 causes osteopetrosis with gingival hamartomas in cattle
    Arnaud Sartelet
    Unit of Animal Genomics, GIGA R and Faculty of Veterinary Medicine, University of Liège B34, 1 Avenue de l Hopital, 4000 Liege Sart Tilman, Belgium
    Dis Model Mech 7:119-28. 2014
  2. 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
  3. 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
  4. ncbi request reprint Endosomal chloride-proton exchange rather than chloride conductance is crucial for renal endocytosis
    Gaia Novarino
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, 13125 Berlin, Germany
    Science 328:1398-401. 2010
  5. doi request reprint Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7
    Lena Wartosch
    Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, Robert Rossle Str 10, D 13125 Berlin, Germany
    FASEB J 23:4056-68. 2009
  6. pmc The late endosomal ClC-6 mediates proton/chloride countertransport in heterologous plasma membrane expression
    Ioana Neagoe
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin, D 13125 Berlin, Germany
    J Biol Chem 285:21689-97. 2010
  7. pmc Role of ClC-5 in renal endocytosis is unique among ClC exchangers and does not require PY-motif-dependent ubiquitylation
    Gesa Rickheit
    Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, Robert Rossle Strasse 10, D 13125 Berlin, Germany
    J Biol Chem 285:17595-603. 2010
  8. pmc Residues important for nitrate/proton coupling in plant and mammalian CLC transporters
    Eun Yeong Bergsdorf
    Department of Physiology and Pathology of Ion Transport, Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, D 13125 Berlin, Germany
    J Biol Chem 284:11184-93. 2009
  9. pmc ClC-7 is a slowly voltage-gated 2Cl(-)/1H(+)-exchanger and requires Ostm1 for transport activity
    Lilia Leisle
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    EMBO J 30:2140-52. 2011
  10. doi request reprint Cell biology and physiology of CLC chloride channels and transporters
    Tobias Stauber
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    Compr Physiol 2:1701-44. 2012

Collaborators

  • R Steinfeld
  • Stephan M Huber
  • Willy Günther
  • Seth L Alper
  • Chris I De Zeeuw
  • Anselm A Zdebik
  • Pawel Fidzinski
  • Lena Wartosch
  • Tom Druet
  • Damien J Keating
  • Martijn Schonewille
  • Gabriele M Rune
  • Tobias Stauber
  • Moritz Hentschke
  • M Pusch
  • Florian Lang
  • Sara Mole
  • Karl S Lang
  • Christian A Hübner
  • Gesa Rickheit
  • Stefanie Weinert
  • Hannes Maier
  • Marco B Rust
  • Raúl Estévez
  • Michaela Schweizer
  • Olaf Scheel
  • Guillermo Spitzmaul
  • Gaia Novarino
  • York Rudhard
  • Ioana Neagoe
  • Eun Yeong Bergsdorf
  • Uwe Kornak
  • Tanja Maritzen
  • Johanna Schleifenbaum
  • Arnaud Sartelet
  • Matthias Heidenreich
  • Mallorie Poet
  • Jens C Fuhrmann
  • Valentin Stein
  • Carmen F Ludwig
  • Patricia Seja
  • Thomas Boettger
  • Gwendolyn M Billig
  • Lilia Leisle
  • Carsten K Pfeffer
  • Vanessa Plans
  • Adrian Muenscher
  • Judith Blanz
  • Philipp F Lange
  • Tatjana Kharkovets
  • Anja Schmitt
  • Rosa Planells-Cases
  • Dagmar Kasper
  • Heimo Ehmke
  • Jörg Faulhaber
  • Rudolf Leuwer
  • Carsten Pfeffer
  • Sabrina Jabs
  • Maik Gollasch
  • Michael Bader
  • Naima Ahariz
  • Wing Lee Chan
  • Yoland Marie Anistan
  • Wouter Coppieters
  • Corinne Fasquelle
  • Natalia Alenina
  • Nadine Cambisano
  • Asif R Pathan
  • István András Szijártó
  • Jean Yves Tano
  • Svea Hohensee
  • Zhiyan Zhang
  • Mario Kassmann
  • Hantz C Hercule
  • Carole Charlier
  • Nancy J Rusch
  • Maarten A Frens
  • Beerend H J Winkelman
  • Leonardo Tolosa
  • Christian Chabbert
  • William Wisden
  • Aleksandra Badura
  • Ilse Klein
  • Florian A Wagner
  • Balázs Pál
  • Sandra M Stobrawa
  • Sven Schaffer
  • Ilka Rinke
  • Nicola Strenzke
  • Corina E Andreescu

Detail Information

Publications45

  1. pmc A missense mutation accelerating the gating of the lysosomal Cl-/H+-exchanger ClC-7/Ostm1 causes osteopetrosis with gingival hamartomas in cattle
    Arnaud Sartelet
    Unit of Animal Genomics, GIGA R and Faculty of Veterinary Medicine, University of Liège B34, 1 Avenue de l Hopital, 4000 Liege Sart Tilman, Belgium
    Dis Model Mech 7:119-28. 2014
    ..These data provide first evidence that accelerated ClC-7/Ostm1 gating per se is deleterious, highlighting a physiological importance of the slow voltage-activation of ClC-7/Ostm1 in lysosomal function and bone resorption. ..
  2. 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...
  3. 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
    ....
  4. ncbi request reprint Endosomal chloride-proton exchange rather than chloride conductance is crucial for renal endocytosis
    Gaia Novarino
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, 13125 Berlin, Germany
    Science 328:1398-401. 2010
    ..However, their proximal tubular endocytosis was also impaired. Thus, endosomal chloride concentration, which is raised by ClC-5 in exchange for protons accumulated by the H+-ATPase, may play a role in endocytosis...
  5. doi request reprint Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7
    Lena Wartosch
    Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, Robert Rossle Str 10, D 13125 Berlin, Germany
    FASEB J 23:4056-68. 2009
    ..These experiments demonstrate that lysosomal pathology is a cell-autonomous consequence of ClC-7 disruption and that ClC-7 is important for lysosomal protein degradation...
  6. pmc The late endosomal ClC-6 mediates proton/chloride countertransport in heterologous plasma membrane expression
    Ioana Neagoe
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin, D 13125 Berlin, Germany
    J Biol Chem 285:21689-97. 2010
    ..Additionally changing the chloride-coordinating serine 157 to proline increased the NO(3)(-) conductance of this mutant. Taken together, these data demonstrate for the first time that ClC-6 is a Cl(-)/H(+) antiporter...
  7. pmc Role of ClC-5 in renal endocytosis is unique among ClC exchangers and does not require PY-motif-dependent ubiquitylation
    Gesa Rickheit
    Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, Robert Rossle Strasse 10, D 13125 Berlin, Germany
    J Biol Chem 285:17595-603. 2010
    ..We conclude that ClC-5 is unique among CLC proteins in being crucial for proximal tubular endocytosis and that PY-motif-dependent ubiquitylation of ClC-5 is dispensable for this role...
  8. pmc Residues important for nitrate/proton coupling in plant and mammalian CLC transporters
    Eun Yeong Bergsdorf
    Department of Physiology and Pathology of Ion Transport, Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, D 13125 Berlin, Germany
    J Biol Chem 284:11184-93. 2009
    ..Gating and proton glutamates play similar roles in bacterial, plant, and mammalian CLC anion/proton exchangers...
  9. pmc ClC-7 is a slowly voltage-gated 2Cl(-)/1H(+)-exchanger and requires Ostm1 for transport activity
    Lilia Leisle
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    EMBO J 30:2140-52. 2011
    ..Our work suggests that gating underlies the rectification of all endosomal/lysosomal CLCs and extends the concept of voltage gating beyond channels to ion exchangers...
  10. doi request reprint Cell biology and physiology of CLC chloride channels and transporters
    Tobias Stauber
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    Compr Physiol 2:1701-44. 2012
    ..The important functions of CLC Cl(-) channels were also revealed by human diseases and mouse models, with phenotypes including myotonia, renal loss of salt and water, deafness, blindness, leukodystrophy, and male infertility...
  11. pmc Vestibular role of KCNQ4 and KCNQ5 K+ channels revealed by mouse models
    Guillermo Spitzmaul
    Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, 13125 Berlin, Germany
    J Biol Chem 288:9334-44. 2013
    ..The impact of postsynaptic KCNQ4 on vestibular function may be related to K(+) removal and modulation of synaptic transmission...
  12. doi request reprint Physiological roles of CLC Cl(-)/H (+) exchangers in renal proximal tubules
    Vanessa Plans
    Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, Berlin, Germany
    Pflugers Arch 458:23-37. 2009
    ..Loss of ClC-7 or its beta-subunit Ostm1 entails lysosomal storage in the PT, in addition to the neuronal lysosomal storage and osteopetrosis that are the hallmarks of ClC-7/Ostm1 loss in mice and men...
  13. pmc Raising cytosolic Cl- in cerebellar granule cells affects their excitability and vestibulo-ocular learning
    Patricia Seja
    Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, Berlin, Germany
    EMBO J 31:1217-30. 2012
    ..These functions, however, were affected by disruption of Kcc2 in PCs. GC excitability plays a previously unknown, but specific role in consolidation of phase learning...
  14. doi request reprint NKCC1-dependent GABAergic excitation drives synaptic network maturation during early hippocampal development
    Carsten K Pfeffer
    Max Delbrück Centrum für Molekulare Medizin MDC and Leibniz Institut für Molekulare Pharmakologie FMP, D 13125 Berlin, Germany
    J Neurosci 29:3419-30. 2009
    ..These data show that NKCC1-mediated Cl(-) accumulation contributes to GABAergic excitation and network activity during early postnatal development and thus facilitates the maturation of excitatory and inhibitory synapses...
  15. 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...
  16. doi request reprint Ca2+-activated Cl− currents are dispensable for olfaction
    Gwendolyn M Billig
    Leibniz Institut für Molekulare Pharmakologie FMP Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    Nat Neurosci 14:763-9. 2011
    ..In contrast with the current view, cyclic nucleotide-gated cation channels do not need a boost by Cl(-) channels to achieve near-physiological levels of olfaction...
  17. ncbi request reprint Stretch-Activation of Angiotensin II Type 1a Receptors Contributes to the Myogenic Response of Mouse Mesenteric and Renal Arteries
    Johanna Schleifenbaum
    From the Medical Clinic for Nephrology and Internal Intensive Care, Charite Campus Virchow Klinikum, Experimental and Clinical Research Center ECRC, Berlin, Germany J S, M K, I A S, H C H, J Y T, Y M A, M G Max Delbrück Center for Molecular Medicine, Berlin, Germany S W, M H, N A, M B, T J J Department Physiology and Pathology of Ion Transport, Leibniz Institut fur Molekulare Pharmakologie FMP, Berlin, Germany S W, M H, T J J Department of Pharmacology and Toxicology, University of Arkansas for Medical Sciences, Little Rock A R P, N J R and Broad Institute of MIT and Harvard, Cambridge, MA M H
    Circ Res 115:263-72. 2014
    ..Vascular wall stretch is the major stimulus for the myogenic response of small arteries to pressure. The molecular mechanisms are elusive, but recent findings suggest that G protein-coupled receptors can elicit a stretch response...
  18. ncbi request reprint Transport activity and presence of ClC-7/Ostm1 complex account for different cellular functions
    Stefanie Weinert
    Leibniz Institut fur Molekulare Pharmakologie FMP, Berlin, Germany Max Delbrück Centrum für Molekulare Medizin MDC, Berlin, Germany
    EMBO Rep 15:784-91. 2014
    ..Our in vivo structure-function analysis of ClC-7 reveals that both protein-protein interactions and ion transport must be considered in the pathogenesis of ClC-7-related diseases. ..
  19. 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
    ....
  20. 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...
  21. 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...
  22. 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...
  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 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...
  25. 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...
  26. 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...
  27. 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...
  28. 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...
  29. 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...
  30. 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...
  31. 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...
  32. 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...
  33. 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...
  34. 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
    ....
  35. 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...
  36. 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...
  37. 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...
  38. 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...
  39. 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...
  40. 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...
  41. 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...
  42. 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...
  43. 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...
  44. 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
    ....
  45. ncbi request reprint Chloride channels are different
    Thomas J Jentsch
    Nature 415:276-7. 2002