[Translate to Deutsch:] Portrait Thomas Jentsch

Prof. Dr. Dr.

Thomas Jentsch

My lab is broadly interested in ion transport processes. Our research spans the whole spectrum from newly identifying ‘novel’ ion channels to studying how their structure determines biophysical transport properties, to determining their role in cellular and organismal processes. Besides plasma membrane channels, we elucidate the roles of ion transport in intracellular organelles. A strong focus is on the role of ion transport in physiology
and disease, which we address with genetic mouse models and human genetics.

We are particularly interested in anion channels. We discovered, for the first time, the CLC family of anion channels and transporters (1990), the molecular identity of volume-regulated VRAC/LRRC8 anion channels (2014), and of acid-activated ASOR/TMEM206 Cl channels (2019). We discovered and/or analyzed the roles of CLCs in myotonia, kidney stones, osteopetrosis, neurodegeneration, leukodystrophy, blindness, deafness, renal salt loss and hyperaldosteronism.

We also discovered all four neuronal Kv7 K channels (KCNQ2-KCNQ5), their role in epilepsy and deafness, and studied the (patho-)physiology of the K-Cl cotransporters KCC2, -3, and -4 using mouse models.

Auszeichnungen

Wilhelm-Vaillant-Preis for biomedical research
1992

Gottfried Wilhelm Leibniz Prize of the Deutsche Forschungsgemeinschaft (DFG)
1995

Alfred Hauptmann Preis for research on epilepsy
1998

Franz Volhard Preis for research in nephrology
1998

International Prize for Translational Neuroscience of the Gertrud-Reemtsma Foundation
1999

Feldberg Prize of the Feldberg Foundation for Anglo-German Scientific Exchange
2000

Familie Hansen Preis
2000

Prix Louis-Jeantet de médecine
2000

Elected Member of the Academia Europaea
2000

Elected Member of EMBO
2000

Ernst-Jung Preis für Medizin
2001

Elected member of the Berlin-Brandenburg Academy of Sciences and Humanities
2001

'Berliner Professorship' Lecture, Yale University
2003

Carl W. Gottschalk Distinguished Lectureship (American Physiological Society)
2004

Adolf-Fick-Prize for Physiology
2004

Elected Member of the Deutsche Akademie der Naturforscher Leopoldina/National Academy of Science
2004

Homer W. Smith Award (American Society of Nephrology)
2004

Founding member of the Akademie der Wissenschaften in Hamburg
2006

Hodgkin-Huxley-Katz Prize Lecture (Physiological Society, UK)
2006

European Research Council (ERC) Advanced Investigator
2011

Hans Ussing Award Lecture (American Physiological Society)
2012

Cátedra de Investigación Dr. García-Sainz, Univ. Autón. San Luís Potosí
2013

Awarded second European Research Council (ERC) Advanced Grant
2017

Honorary Degree (Dr. h.c.) by Medical Faculty of Hamburg University
2017

Science Prize 'Society needs Science' (by Stifterverband/Leibniz Society)
2018

CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease

  • Thomas J. Jentsch; Michael Pusch

Physiological Reviews 2018

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A gain-of-function mutation in the CLCN2 chloride channel gene causes primary aldosteronism

  • Fernandes-Rosa, F.L.; Daniil, G.; Orozco, I.J.; Göppner, C.; El Zein, R.; Jain, V.; Boulkroun, S.; Jeunemaitre, X.; Amar, L.; Lefebvre, H.; Schwarzmayr, T.; Strom, T.M.; Jentsch, T.J.; Zennaro, M.-C.

Nature Genetics 2018

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LRRC8/VRAC anion channels enhance β-cell glucose sensing and insulin secretion

  • Till Stuhlmann; Rosa Planells-Cases; Thomas J. Jentsch

Nature Communications 2018

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K <inf>2P</inf> TASK-2 and KCNQ1–KCNE3 K <sup>+</sup> channels are major players contributing to intestinal anion and fluid secretion

  • Julio-Kalajzić, F.; Villanueva, S.; Burgos, J.; Ojeda, M.; Cid, L.P.; Jentsch, T.J.; Sepúlveda, F.V.

Journal of Physiology 2018

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Reply from L. P. Cid, T. J. Jentsch and F. V. Sepúlveda: intestinal electrolyte and fluid secretion – a model in trouble?

  • Cid, L.P.; Jentsch, T.J.; Sepúlveda, F.V.

Journal of Physiology 2018

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De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females

  • Palmer, E.E.; Stuhlmann, T.; Weinert, S.; Haan, E.; Van Esch, H.; Holvoet, M.; Boyle, J.; Leffler, M.; Raynaud, M.; Moraine, C.; Van Bokhoven, H.; Kleefstra, T.; Kahrizi, K.; Najmabadi, H.; Ropers, H.-H.; Delgado, M.R.; Sirsi, D.; Golla, S.; Sommer, A.; Pietryga, M.P.; Chung, W.K.; Wynn, J.; Rohena, L.; Bernardo, E.; Hamlin, D.; Faux, B.M.; Grange, D.K.; Manwaring, L.; Tolmie, J.; Joss, S.; Study, D.D.D.; Cobben, J.M.; Duijkers, F.A.M.; Goehringer, J.M.; Challman, T.D.; Hennig, F.; Fischer, U.; Grimme, A.; Suckow, V.; Musante, L.; Nicholl, J.; Shaw, M.; Lodh, S.P.; Niu, Z.; Rosenfeld, J.A.; Stankiewicz, P.; Jentsch, T.J.; Gecz, J.; Field, M.; Kalscheuer, V.M.

Molecular Psychiatry 2018

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Ca2+-activated Cl−currents in the murine vomeronasal organ enhance neuronal spiking but are dispensable for male-male aggression

  • Jonas Münch; Gwendolyn Billig; Christian A. Huebner; Trese Leinders-Zufall; Frank Zufall; Thomas J. Jentsch

Journal of Biological Chemistry 2018

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LRRC8/VRAC anion channels are required for late stages of spermatid development in mice

  • Jennifer C. Lück; Dmytro Puchkov; Florian Ullrich; Thomas J. Jentsch

Journal of Biological Chemistry 2018

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LRRC8 N termini influence pore properties and gating of volume-regulated anion channels (VRACs)

  • Pingzheng Zhou; Maya M. Polovitskaya; Thomas J. Jentsch

Journal of Biological Chemistry 2018

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De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females.

  • Palmer EE; Stuhlmann T; Weinert S; Haan E; Van Esch H; Holvoet M; Boyle J; Leffler M; Raynaud M; Moraine C; van Bokhoven H; Kleefstra T; Kahrizi K; Najmabadi H; Ropers HH; Delgado MR; Sirsi D; Golla S; Sommer A; Pietryga MP
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Disruption of Kcc2-dependent inhibition of olfactory bulb output neurons suggests its importance in odour discrimination.

  • Gödde K; Gschwend O; Puchkov D; Pfeffer CK; Carleton A; Jentsch TJ
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The ClC-K2 Chloride Channel Is Critical for Salt Handling in the Distal Nephron.

  • Hennings JC; Andrini O; Picard N; Paulais M; Huebner AK; Cayuqueo IK; Bignon Y; Keck M; Cornière N; Böhm D; Jentsch TJ; Chambrey R; Teulon J; Hübner CA; Eladari D
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Inactivation and Anion Selectivity of Volume-Regulated VRAC Channels Depend on Carboxy-Terminal Residues of the First Extracellular Loop.

  • Ullrich F; Reincke SM; Voss FK; Stauber T; Jentsch TJ
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VRACs and other ion channels and transporters in the regulation of cell volume and beyond.

  • Jentsch TJ
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Inactivation and anion selectivity of volume-regulated anion channels (VRACs) depend on c-terminal residues of the first extracellular loop

  • Ullrich, F.; Reincke, S.M.; Voss, F.K.; Stauber, T.; Jentsch, T.J.

Journal of Biological Chemistry 2016

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KCNQ Potassium Channels Modulate Sensitivity of Skin Down-hair (D-hair) Mechanoreceptors.

  • Schütze S; Orozco IJ; Jentsch TJ
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VRAC: molecular identification as LRRC8 heteromers with differential functions.

  • Jentsch TJ; Lutter D; Planells-Cases R; Ullrich F; Voss FK
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X-exome sequencing of 405 unresolved families identifies seven novel intellectual disability genes.

  • Hu H; Haas SA; Chelly J; Van Esch H; Raynaud M; de Brouwer AP; Weinert S; Froyen G; Frints SG; Laumonnier F; Zemojtel T; Love MI; Richard H; Emde AK; Bienek M; Jensen C; Hambrock M; Fischer U; Langnick C; Feldkamp M
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Stretch-activation of angiotensin II type 1a receptors contributes to the myogenic response of mouse mesenteric and renal arteries.

  • Schleifenbaum J; Kassmann M; Szijártó IA; Hercule HC; Tano JY; Weinert S; Heidenreich M; Pathan AR; Anistan YM; Alenina N; Rusch NJ; Bader M; Jentsch TJ; Gollasch M
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Transport activity and presence of ClC-7/Ostm1 complex account for different cellular functions.

  • Weinert S; Jabs S; Hohensee S; Chan WL; Kornak U; Jentsch TJ
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Identification of LRRC8 heteromers as an essential component of the volume-regulated anion channel VRAC.

  • Voss FK; Ullrich F; Münch J; Lazarow K; Lutter D; Mah N; Andrade-Navarro MA; von Kries JP; Stauber T; Jentsch TJ
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CLCN7 and TCIRG1 mutations differentially affect bone matrix mineralization in osteopetrotic individuals.

  • Barvencik F; Kurth I; Koehne T; Stauber T; Zustin J; Tsiakas K; Ludwig CF; Beil FT; Pestka JM; Hahn M; Santer R; Supanchart C; Kornak U; Del Fattore A; Jentsch TJ; Teti A; Schulz A; Schinke T; Amling M
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Disrupting MLC1 and GlialCAM and ClC-2 interactions in leukodystrophy entails glial chloride channel dysfunction.

  • Hoegg-Beiler MB; Sirisi S; Orozco IJ; Ferrer I; Hohensee S; Auberson M; Gödde K; Vilches C; de Heredia ML; Nunes V; Estévez R; Jentsch TJ
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A missense mutation accelerating the gating of the lysosomal Cl-/H+-exchanger ClC-7/Ostm1 causes osteopetrosis with gingival hamartomas in cattle.

  • Sartelet A; Stauber T; Coppieters W; Ludwig CF; Fasquelle C; Druet T; Zhang Z; Ahariz N; Cambisano N; Jentsch TJ; Charlier C
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ClC-7 expression levels critically regulate bone turnover, but not gastric acid secretion.

  • Supanchart C; Wartosch L; Schlack C; Kühnisch J; Felsenberg D; Fuhrmann JC; de Vernejoul MC; Jentsch TJ; Kornak U
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The CLC family of chloride channels and transporters

  • Stauber, T.; Novarino, G.; Jentsch, T.J.

Physiology and Pathology of chloride transporters and channels in the nervous system 2010

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Sorting motifs of the endosomal/lysosomal CLC chloride transporters.

  • Stauber T; Jentsch TJ
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A cation counterflux supports lysosomal acidification.

  • Steinberg BE; Huynh KK; Brodovitch A; Jabs S; Stauber T; Jentsch TJ; Grinstein S
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Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation.

  • Weinert S; Jabs S; Supanchart C; Schweizer M; Gimber N; Richter M; Rademann J; Stauber T; Kornak U; Jentsch TJ
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The KCNQ5 potassium channel mediates a component of the afterhyperpolarization current in mouse hippocampus.

  • Tzingounis AV; Heidenreich M; Kharkovets T; Spitzmaul G; Jensen HS; Nicoll RA; Jentsch TJ
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The late endosomal ClC-6 mediates proton/chloride countertransport in heterologous plasma membrane expression.

  • Neagoe I; Stauber T; Fidzinski P; Bergsdorf EY; Jentsch TJ
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Endosomal chloride-proton exchange rather than chloride conductance is crucial for renal endocytosis.

  • Novarino G; Weinert S; Rickheit G; Jentsch TJ
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Analysis of CLCN2 as candidate gene for megalencephalic leukoencephalopathy with subcortical cysts.

  • Scheper GC; van Berkel CG; Leisle L; de Groot KE; Errami A; Jentsch TJ; Van der Knaap MS
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Role of ClC-5 in renal endocytosis is unique among ClC exchangers and does not require PY-motif-dependent ubiquitylation.

  • Rickheit G; Wartosch L; Schaffer S; Stobrawa SM; Novarino G; Weinert S; Jentsch TJ
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Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport.

  • Preston P; Wartosch L; Günzel D; Fromm M; Kongsuphol P; Ousingsawat J; Kunzelmann K; Barhanin J; Warth R; Jentsch TJ
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No evidence for a role of CLCN2 variants in idiopathic generalized epilepsy.

  • Niemeyer MI; Cid LP; Sepúlveda FV; Blanz J; Auberson M; Jentsch TJ
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Distinct neuropathologic phenotypes after disrupting the chloride transport proteins ClC-6 or ClC-7/Ostm1.

  • Pressey SN; O'Donnell KJ; Stauber T; Fuhrmann JC; Tyynelä J; Jentsch TJ; Cooper JD
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ClC-3--a granular anion transporter involved in insulin secretion?

  • Jentsch TJ; Maritzen T; Keating DJ; Zdebik AA; Thévenod F
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Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.

  • Poët M; Kornak U; Schweizer M; Zdebik AA; Scheel O; Hoelter S; Wurst W; Schmitt A; Fuhrmann JC; Planells-Cases R; Mole SE; Hübner CA; Jentsch TJ
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Kidney-specific upregulation of vitamin D3 target genes in ClC-5 KO mice.

  • Maritzen T; Rickheit G; Schmitt A; Jentsch TJ
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Structural determinants of M-type KCNQ (Kv7) K+ channel assembly.

  • Schwake M; Athanasiadu D; Beimgraben C; Blanz J; Beck C; Jentsch TJ; Saftig P; Friedrich T
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ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function.

  • Lange PF; Wartosch L; Jentsch TJ; Fuhrmann JC
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Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness.

  • Kharkovets T; Dedek K; Maier H; Schweizer M; Khimich D; Nouvian R; Vardanyan V; Leuwer R; Moser T; Jentsch TJ
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Neurogenic mechanisms contribute to hypertension in mice with disruption of the K-Cl cotransporter KCC3.

  • Rust MB; Faulhaber J; Budack MK; Pfeffer C; Maritzen T; Didié M; Beck FX; Boettger T; Schubert R; Ehmke H; Jentsch TJ; Hübner CA
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Mice with a targeted disruption of the Cl-/HCO3- exchanger AE3 display a reduced seizure threshold.

  • Hentschke M; Wiemann M; Hentschke S; Kurth I; Hermans-Borgmeyer I; Seidenbecher T; Jentsch TJ; Gal A; Hübner CA
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Physiological Functions of the CLC Chloride Transport Proteins

  • Maritzen, T.; Blanz, J.; Jentsch, T.

Advances in Molecular and Cell Biology 2006

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Disruption of KCC2 reveals an essential role of K-Cl cotransport already in early synaptic inhibition.

  • Hübner CA; Stein V; Hermans-Borgmeyer I; Meyer T; Ballanyi K; Jentsch TJ
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Mutations in CAV3 cause mechanical hyperirritability of skeletal muscle in rippling muscle disease.

  • Betz RC; Schoser BG; Kasper D; Ricker K; Ramírez A; Stein V; Torbergsen T; Lee YA; Nöthen MM; Wienker TF; Malin JP; Propping P; Reis A; Mortier W; Jentsch TJ; Vorgerd M; Kubisch C
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Barttin is a Cl- channel beta-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion.

  • Estévez R; Boettger T; Stein V; Birkenhäger R; Otto E; Hildebrandt F; Jentsch TJ
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Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel.

  • Dedek K; Kunath B; Kananura C; Reuner U; Jentsch TJ; Steinlein OK
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Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption.

  • Bösl MR; Stein V; Hübner C; Zdebik AA; Jordt SE; Mukhopadhyay AK; Davidoff MS; Holstein AF; Jentsch TJ
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Tissue distribution and subcellular localization of the ClC-5 chloride channel in rat intestinal cells.

  • Vandewalle A; Cluzeaud F; Peng KC; Bens M; Lüchow A; Günther W; Jentsch TJ
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Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man.

  • Kornak U; Kasper D; Bösl MR; Kaiser E; Schweizer M; Schulz A; Friedrich W; Delling G; Jentsch TJ
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Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus.

  • Stobrawa SM; Breiderhoff T; Takamori S; Engel D; Schweizer M; Zdebik AA; Bösl MR; Ruether K; Jahn H; Draguhn A; Jahn R; Jentsch TJ
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Tissue distribution and subcellular localization of the C1C-5 chloride channel in rat intestinal cells

  • Vandewalle, A.; Cluzeaud, F.; Peng, K.-C.; Bens, M.; Lüchow, A.; Günther, W.; Jentsch, T.J.

American Journal of Physiology - Cell Physiology 2001

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Planned law could put German research at risk [4]

  • Jentsch, T.

Nature 2001

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An internalization signal in ClC-5, an endosomal Cl-channel mutated in dent's disease.

  • Schwake M; Friedrich T; Jentsch TJ
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Pores formed by single subunits in mixed dimers of different CLC chloride channels.

  • Weinreich F; Jentsch TJ
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A constitutively open potassium channel formed by KCNQ1 and KCNE3.

  • Schroeder BC; Waldegger S; Fehr S; Bleich M; Warth R; Greger R; Jentsch TJ
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Pathophysiology of KCNQ channels: neonatal epilepsy and progressive deafness.

  • Jentsch TJ; Schroeder BC; Kubisch C; Friedrich T; Stein V
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Mutations in the a3 subunit of the vacuolar H(+)-ATPase cause infantile malignant osteopetrosis.

  • Kornak U; Schulz A; Friedrich W; Uhlhaas S; Kremens B; Voit T; Hasan C; Bode U; Jentsch TJ; Kubisch C
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Functional and structural analysis of ClC-K chloride channels involved in renal disease.

  • Waldegger S; Jentsch TJ
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Inhibition of KCNQ1-4 potassium channels expressed in mammalian cells via M1 muscarinic acetylcholine receptors.

  • Selyanko AA; Hadley JK; Wood IC; Abogadie FC; Jentsch TJ; Brown DA
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ClC-5 Cl- -channel disruption impairs endocytosis in a mouse model for Dent's disease.

  • Piwon N; Günther W; Schwake M; Bösl MR; Jentsch TJ
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Neuronal KCNQ potassium channels: physiology and role in disease.

  • Jentsch TJ
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KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.

  • Kharkovets T; Hardelin JP; Safieddine S; Schweizer M; El-Amraoui A; Petit C; Jentsch TJ
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KCNQ5, a novel potassium channel broadly expressed in brain, mediates M-type currents.

  • Schroeder BC; Hechenberger M; Weinreich F; Kubisch C; Jentsch TJ
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Characterization of renal chloride channel (CLCN5) mutations in Dent's disease.

  • Yamamoto K; Cox JP; Friedrich T; Christie PT; Bald M; Houtman PN; Lapsley MJ; Patzer L; Tsimaratos M; Van'T Hoff WG; Yamaoka K; Jentsch TJ; Thakker RV
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From tonus to tonicity: physiology of CLC chloride channels.

  • Waldegger S; Jentsch TJ
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Neurological diseases caused by ion-channel mutations.

  • Weinreich F; Jentsch TJ
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Surface expression and single channel properties of KCNQ2/KCNQ3, M-type K+ channels involved in epilepsy.

  • Schwake M; Pusch M; Kharkovets T; Jentsch TJ
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Mutations in the a3 subunit of the vacuolar H<sup>+</sup>-ATPase cause infantile malignant osteopetrosis

  • Kornak, U.; Schulz, A.; Friedrich, W.; Uhlhaas, S.; Kremens, B.; Voit, T.; Hasan, C.; Bode, U.; Jentsch, T.J.; Kubisch, C.

Human Molecular Genetics 2000

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From tonus to tonicity: Physiology of CLC chloride channels

  • Waldegger, S.; Jentsch, T.J.

Journal of the American Society of Nephrology 2000

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Characterization of renal chloride channel (CLCN5) mutations in Dent's disease

  • Yamamoto, K.; Cox, J.P.D.T.; Friedrich, T.; Christie, P.T.; Bald, M.; Houtman, P.N.; Lapsley, M.J.; Patzer, L.; Tsimaratos, M.; Van't Hoff, W.G.; Yamaoka, K.; Jentsch, T.J.; Thakker, R.V.

Journal of the American Society of Nephrology 2000

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Moderate loss of function of cyclic-AMP-modulated KCNQ2/KCNQ3 K+ channels causes epilepsy.

  • Schroeder BC; Kubisch C; Stein V; Jentsch TJ
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Functional characterization of renal chloride channel, CLCN5, mutations associated with Dent'sJapan disease.

  • Igarashi T; Günther W; Sekine T; Inatomi J; Shiraga H; Takahashi S; Suzuki J; Tsuru N; Yanagihara T; Shimazu M; Jentsch TJ; Thakker RV
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ClC-1 chloride channel mutations in myotonia congenita: variable penetrance of mutations shifting the voltage dependence.

  • Kubisch C; Schmidt-Rose T; Fontaine B; Bretag AH; Jentsch TJ
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Golgi localization and functionally important domains in the NH2 and COOH terminus of the yeast CLC putative chloride channel Gef1p.

  • Schwappach B; Stobrawa S; Hechenberger M; Steinmeyer K; Jentsch TJ
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Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions.

  • Rychkov GY; Pusch M; Roberts ML; Jentsch TJ; Bretag AH
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Determinants of slow gating in ClC-0, the voltage-gated chloride channel of Torpedo marmorata.

  • Fong P; Rehfeldt A; Jentsch TJ
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Novel muscle chloride channel (CLCN1) mutations in myotonia congenita with various modes of inheritance including incomplete dominance and penetrance.

  • Plassart-Schiess E; Gervais A; Eymard B; Lagueny A; Pouget J; Warter JM; Fardeau M; Jentsch TJ; Fontaine B
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Molecular diagnosis of McArdle disease: revised genomic structure of the myophosphorylase gene and identification of a novel mutation.

  • Kubisch C; Wicklein EM; Jentsch TJ
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KCNQ2, the first gene found to be mutated in human generalized idiopathic epilepsy,Le KCNQ2 est le premier gene pour lequel une mutation est demontree chez des patients souffrant d'epilepsie generalisee idiopathique

  • Steinlein, O.K.; Jentsch, T.J.

Pathologie Biologie 1998

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Determinants of slow gating in ClC-0, the voltage-gated chloride channel of Torpedo marmorata

  • Fong, P.; Rehfeldt, A.; Jentsch, T.J.

American Journal of Physiology - Cell Physiology 1998

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Molecular physiology of renal chloride channels.

  • Steinmeyer K; Jentsch TJ
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ClC-5, the chloride channel mutated in Dent's disease, colocalizes with the proton pump in endocytotically active kidney cells.

  • Günther W; Lüchow A; Cluzeaud F; Vandewalle A; Jentsch TJ
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Characterization of the hyperpolarization-activated chloride current in dissociated rat sympathetic neurons.

  • Clark S; Jordt SE; Jentsch TJ; Mathie A
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A potassium channel mutation in neonatal human epilepsy.

  • Biervert C; Schroeder BC; Kubisch C; Berkovic SF; Propping P; Jentsch TJ; Steinlein OK
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CLC chloride channels in human disease

  • Jentsch, T.J.

FASEB Journal 1997

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Localization and induction by dehydration of ClC-K chloride channels in the rat kidney

  • Vandewalle, A.; Cluzeaud, F.; Bens, M.; Kieferle, S.; Steinmeyer, K.; Jentsch, T.J.

American Journal of Physiology - Renal Physiology 1997

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Molecular dissection of gating in the ClC-2 chloride channel.

  • Jordt SE; Jentsch TJ
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Pathophysiological mechanisms of dominant and recessive KVLQT1 K+ channel mutations found in inherited cardiac arrhythmias.

  • Wollnik B; Schroeder BC; Kubisch C; Esperer HD; Wieacker P; Jentsch TJ
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Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders.

  • Lloyd SE; Gunther W; Pearce SH; Thomson A; Bianchi ML; Bosio M; Craig IW; Fisher SE; Scheinman SJ; Wrong O; Jentsch TJ; Thakker RV
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Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1.

  • Schmidt-Rose T; Jentsch TJ
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Idiopathic low molecular weight proteinuria associated with hypercalciuric nephrocalcinosis in Japanese children is due to mutations of the renal chloride channel (CLCN5).

  • Lloyd SE; Pearce SH; Günther W; Kawaguchi H; Igarashi T; Jentsch TJ; Thakker RV
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Independent gating of single pores in CLC-0 chloride channels.

  • Ludewig U; Pusch M; Jentsch TJ
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Inward rectification in ClC-0 chloride channels caused by mutations in several protein regions.

  • Ludewig U; Jentsch TJ; Pusch M
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Transmembrane topology of a CLC chloride channel.

  • Schmidt-Rose T; Jentsch TJ
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Localization and induction by dehydration of ClC-K chloride channels in the rat kidney.

  • Vandewalle A; Cluzeaud F; Bens M; Kieferle S; Steinmeyer K; Jentsch TJ
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Analysis of a protein region involved in permeation and gating of the voltage-gated Torpedo chloride channel ClC-0.

  • Ludewig U; Jentsch TJ; Pusch M
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Chloride channels: an emerging molecular picture.

  • Jentsch TJ; Günther W
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Temperature dependence of fast and slow gating relaxations of ClC-0 chloride channels.

  • Pusch M; Ludewig U; Jentsch TJ
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Cloning and functional expression of rat CLC-5, a chloride channel related to kidney disease.

  • Steinmeyer K; Schwappach B; Bens M; Vandewalle A; Jentsch TJ
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Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the CIC-1 chloride channel.

  • Pusch M; Steinmeyer K; Koch MC; Jentsch TJ
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Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCN1) leading to myotonia.

  • Meyer-Kleine C; Steinmeyer K; Ricker K; Jentsch TJ; Koch MC
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Molecular basis of epithelial Cl channels.

  • Fong P; Jentsch TJ
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Gating of the voltage-dependent chloride channel CIC-0 by the permeant anion.

  • Pusch M; Ludewig U; Rehfeldt A; Jentsch TJ
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Myotonias due to CLC-1 chloride channel mutations.

  • Jentsch TJ; Lorenz C; Pusch M; Steinmeyer K
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Properties of voltage-gated chloride channels of the ClC gene family.

  • Jentsch TJ; Günther W; Pusch M; Schwappach B
online lesen

Myotonias due to CLC-1 chloride channel mutations.

  • Jentsch, T.J.; Lorenz, C.; Pusch, M.; Steinmeyer, K.

Society of General Physiologists series 1995

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Myotonias due to CLC-1 chloride channel mutations

  • Jentsch, T.J.; Lorenz, C.; Pusch, M.; Steinmeyer, K.

Journal of General Physiology 1995

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Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCNI) leading to myotonia

  • Meyer-Kleine, C.; Steinmeyer, K.; Ricker, K.; Jentsch, T.J.; Koch, M.C.

American Journal of Human Genetics 1995

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Properties of voltage‐gated chloride channels of the ClC gene family.

  • Jentsch, T.J.; Günther, W.; Pusch, M.; Schwappach, B.

The Journal of Physiology 1995

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Chloride channel 2 gene (Clc2) maps to chromosome 16 of the mouse, extending a region of conserved synteny with human chromosome 3q

  • Lengeling, A.; Gronemeier, M.; Ronsiek, M.; Thiemann, A.; Jentsch, T.J.; Jockusch, H.

Genetical Research 1995

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Chloride channel 2 gene (Clc2) maps to chromosome 16 of the mouse, extending a region of conserved synteny with human chromosome 3q.

  • Lengeling A; Gronemeier M; Ronsiek M; Thiemann A; Jentsch TJ; Jockusch H
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ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family.

  • Brandt S; Jentsch TJ
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Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle.

  • Klocke R; Steinmeyer K; Jentsch TJ; Jockusch H
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Molecular physiology of voltage-gated chloride channels.

  • Pusch M; Jentsch TJ
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Molecular physiology of anion channels.

  • Jentsch TJ
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Two highly homologous members of the ClC chloride channel family in both rat and human kidney.

  • Kieferle S; Fong P; Bens M; Vandewalle A; Jentsch TJ
online lesen

Low single channel conductance of the major skeletal muscle chloride channel, ClC-1.

  • Pusch M; Steinmeyer K; Jentsch TJ
online lesen

Chapter 2 Molecular Biology of Voltage-Gated Chloride Channels

  • Thomas J. Jentsch

Chloride Channels 1994

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Structure and function of ClC chloride channels.

  • Jentsch, T.J.

The Japanese journal of physiology 1994

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Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle

  • Klocke, R.; Steinmeyer, K.; Jentsch, T.J.; Jockusch, H.

Journal of Biological Chemistry 1994

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Nonsense and missense mutations in the muscular chloride channel gene Clc- 1 of myotonic mice

  • Gronemeier, M.; Condie, A.; Prosser, J.; Steinmeyer, K.; Jentsch, T.J.; Jockusch, H.

Journal of Biological Chemistry 1994

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Multimeric structure of CIC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen)

  • Steinmeyer, K.; Lorenz, C.; Pusch, M.; Koch, M.C.; Jentsch, T.J.

EMBO Journal 1994

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Molecular physiology of voltage-gated chloride channels

  • Pusch, M.; Jentsch, T.J.

Physiological Reviews 1994

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Genomic organization of the human muscle chloride channel CIC-1 and analysis of novel mutations leading to Becker-type myotonia.

  • Lorenz C; Meyer-Kleine C; Steinmeyer K; Koch MC; Jentsch TJ
online lesen

Nonsense and missense mutations in the muscular chloride channel gene Clc-1 of myotonic mice.

  • Gronemeier M; Condie A; Prosser J; Steinmeyer K; Jentsch TJ; Jockusch H
online lesen

Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen).

  • Steinmeyer K; Lorenz C; Pusch M; Koch MC; Jentsch TJ
online lesen

Trinity of cation channels.

  • Jentsch TJ
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Kinetic properties of the sodium bicarbonate (carbonate) symport in monkey kidney epithelial cells (BSC-1). Interactions between Na+, HCO-3, and pH.

  • Jentsch TJ; Schwartz P; Schill BS; Langner B; Lepple AP; Keller SK; Wiederholt M
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Electrical properties of sodium bicarbonate symport in kidney epithelial cells (BSC-1).

  • Jentsch TJ; Matthes H; Keller SK; Wiederholt M
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The regulation of intracellular pH in monkey kidney epithelial cells (BSC-1). Roles of Na+/H+ antiport, Na+-HCO3(-)-(NaCO3-) symport, and Cl-/HCO3- exchange.

  • Jentsch TJ; Janicke I; Sorgenfrei D; Keller SK; Wiederholt M
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Interactions of pH and K+ conductance in cultured bovine retinal pigment epithelial cells.

  • Keller SK; Jentsch TJ; Koch M; Wiederholt M
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The regulation of intracellular pH in monkey kidney epithelial cells (BSC-1). Roles of Na<sup>+</sup>/H<sup>+</sup> antiport, Na<sup>+</sup>-HCO<inf>3</inf><sup>-</sup>-(NaCO<inf>3</inf><sup>-</sup>) symport, and Cl<sup>-</sup>/HCO<inf>3</inf><sup>-</sup> exchange

  • Jentsch, T.J.; Janicke, I.; Sorgenfrei, D.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1986

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Kinetic properties of the sodium bicarbonate (carbonate) symport in monkey kidney epithelial cells (BSC-1). Interactins between Na<sup>+</sup>, HCO<inf>3</inf><sup>-</sup>, and pH

  • Jentsch, T.J.; Schwartz, P.; Schill, B.S.; Langner, B.; Lepple, A.P.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1986

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Electrical properties of sodium bicarbonate symport in kidney epithelial cells (BSC-1)

  • Jentsch, T.J.; Matthes, H.; Keller, S.K.; Wiederholt, M.

American Journal of Physiology - Renal Fluid and Electrolyte Physiology 1986

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Interactions of pH and K<sup>+</sup> conductance in cultured bovine retinal pigment epithelial cells

  • Keller, S.K.; Jentsch, T.J.; Koch, M.; Wiederholt, M.

American Journal of Physiology - Cell Physiology 1986

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Kidney epithelial cells of monkey origin (BSC-1) express a sodium bicarbonate cotransport. Characterization by 22Na+ flux measurements.

  • Jentsch TJ; Schill BS; Schwartz P; Matthes H; Keller SK; Wiederholt M
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Observation of doubly charged triatomic cluster ions in field evaporation

  • Drachsel, W.; Jentsch, Th.; Gingerich, K.A.; Block, J.H.

Surface Science 1985

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Ion transport mechanisms in cultured bovine corneal endothelial cells

  • Jentsch, T.J.; Keller, S.K.; Wiederholt, M.

Current Eye Research 1985

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Response of the intracellular potentials of cultured bovine lens cells to ions and inhibitors.

  • Jentsch TJ; von der Haar B; Keller SK; Wiederholt M
online lesen

Ion transport mechanisms in cultured bovine corneal endothelial cells.

  • Jentsch TJ; Keller SK; Wiederholt M
online lesen

Anion dependence of electrical effects of bicarbonate and sodium on cultured bovine corneal endothelial cells.

  • Jentsch TJ; Matthes H; Keller SK; Wiederholt M
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Kidney epithelial cells of monkey origin (BSC-1) express a sodium bicarbonate cotransport. Characterization by <sup>22</sup>Na<sup>+</sup> flux measurements

  • Jentsch, T.J.; Schill, B.S.; Schwartz, P.; Matthes, H.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1985

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A bicarbonate-dependent process inhibitable by disulfonic stilbenes and a Na<sup>+</sup>/H<sup>+</sup> exchange mediate <sup>22</sup>Na<sup>+</sup> uptake into cultured bovine corneal endothelium

  • Jentsch, T.J.; Stahlknecht, T.R.; Hollwede, H.; Fischer, D.G.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1985

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Electrogenic sodium-bicarbonate symport in cultured corneal endothelial cells.

  • Wiederholt M; Jentsch TJ; Keller SK
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A bicarbonate-dependent process inhibitable by disulfonic stilbenes and a Na+/H+ exchange mediate 22Na+ uptake into cultured bovine corneal endothelium.

  • Jentsch TJ; Stahlknecht TR; Hollwede H; Fischer DG; Keller SK; Wiederholt M
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KCNQ5 Controls Perivascular Adipose Tissue–Mediated Vasodilation

  • Dmitry Tsvetkov; Johanna Schleifenbaum; Yibin Wang; Mario Kassmann; Maya M. Polovitskaya; Mohamed Ali; Sebastian Schütze; Michael Rothe; Friedrich C. Luft; Thomas J. Jentsch; Maik Gollasch

Hypertension 2024

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ClC-7 drives intraphagosomal chloride accumulation to support hydrolase activity and phagosome resolution

  • Jing Ze Wu; Mariia Zeziulia; Whijin Kwon; Thomas J. Jentsch; Sergio Grinstein; Spencer A. Freeman

Journal of Cell Biology 2023

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Impaired Autophagic Clearance with a Gain-of-Function Variant of the Lysosomal Cl−/H+ Exchanger ClC-7

  • Shroddha Bose; Cecilia de Heus; Mary E. Kennedy; Fan Wang; Thomas J. Jentsch; Judith Klumperman; Tobias Stauber

Biomolecules 2023

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Gating choreography and mechanism of the human proton-activated chloride channel ASOR

  • Chongyuan Wang; Maya M. Polovitskaya; Bryce D. Delgado; Thomas J. Jentsch; Stephen B. Long

Science Advances 2022

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Proton-gated anion transport governs macropinosome shrinkage

  • Mariia Zeziulia; Sandy Blin; Franziska W. Schmitt; Martin Lehmann; Thomas J. Jentsch

Nature Cell Biology 2022

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Renal Deletion of LRRC8/VRAC Channels Induces Proximal Tubulopathy

  • Karen I. López-Cayuqueo; Rosa Planells-Cases; Matthias Pietzke; Anna Oliveras; Stefan Kempa; Sebastian Bachmann; Thomas J. Jentsch

Journal of the American Society of Nephrology 2022

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Cellular basis of ClC-2 Cl− channel–related brain and testis pathologies

  • Corinna Göppner; Audrey H. Soria; Maja B. Hoegg-Beiler; Thomas J. Jentsch

Journal of Biological Chemistry 2021

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Transfer of cGAMP into Bystander Cells via LRRC8 Volume-Regulated Anion Channels Augments STING-Mediated Interferon Responses and Anti-viral Immunity

  • Thomas Jentsch

Immunity 2020

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Cryo-EM structure of the volume-regulated anion channel LRRC8D isoform identifies features important for substrate permeation

  • Thomas Jentsch

Communications Biology 2020

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A recurrent gain-of-function mutation in CLCN6, encoding the ClC-6 Cl-/H+-exchanger, causes early-onset neurodegeneration

  • Thomas Jentsch
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Pathogenesis of hypertension in a mouse model for human CLCN2 related hyperaldosteronism

  • Corinna Göppner; Ian J. Orozco; Maja B. Hoegg-Beiler; Audrey H. Soria; Christian A. Hübner; Fabio L. Fernandes-Rosa; Sheerazed Boulkroun; Maria-Christina Zennaro; Thomas J. Jentsch

Nature Communications 2019

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Identification of TMEM206 proteins as pore of ASOR acid-sensitive chloride channels

  • Florian Ullrich; Sandy Blin; Katina Lazarow; Tony Daubitz; Jens-Peter von Kries; Thomas J. Jentsch
online lesen

Identification of TMEM206 proteins as pore of PAORAC/ASOR acid-sensitive chloride channels

  • Florian Ullrich; Sandy Blin; Katina Lazarow; Tony Daubitz; Jens Peter von Kries; Thomas J Jentsch

eLife 2019

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CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease

  • Thomas J. Jentsch; Michael Pusch

Physiological Reviews 2018

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A gain-of-function mutation in the CLCN2 chloride channel gene causes primary aldosteronism

  • Fernandes-Rosa, F.L.; Daniil, G.; Orozco, I.J.; Göppner, C.; El Zein, R.; Jain, V.; Boulkroun, S.; Jeunemaitre, X.; Amar, L.; Lefebvre, H.; Schwarzmayr, T.; Strom, T.M.; Jentsch, T.J.; Zennaro, M.-C.

Nature Genetics 2018

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LRRC8/VRAC anion channels enhance β-cell glucose sensing and insulin secretion

  • Till Stuhlmann; Rosa Planells-Cases; Thomas J. Jentsch

Nature Communications 2018

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K <inf>2P</inf> TASK-2 and KCNQ1–KCNE3 K <sup>+</sup> channels are major players contributing to intestinal anion and fluid secretion

  • Julio-Kalajzić, F.; Villanueva, S.; Burgos, J.; Ojeda, M.; Cid, L.P.; Jentsch, T.J.; Sepúlveda, F.V.

Journal of Physiology 2018

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Reply from L. P. Cid, T. J. Jentsch and F. V. Sepúlveda: intestinal electrolyte and fluid secretion – a model in trouble?

  • Cid, L.P.; Jentsch, T.J.; Sepúlveda, F.V.

Journal of Physiology 2018

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De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females

  • Palmer, E.E.; Stuhlmann, T.; Weinert, S.; Haan, E.; Van Esch, H.; Holvoet, M.; Boyle, J.; Leffler, M.; Raynaud, M.; Moraine, C.; Van Bokhoven, H.; Kleefstra, T.; Kahrizi, K.; Najmabadi, H.; Ropers, H.-H.; Delgado, M.R.; Sirsi, D.; Golla, S.; Sommer, A.; Pietryga, M.P.; Chung, W.K.; Wynn, J.; Rohena, L.; Bernardo, E.; Hamlin, D.; Faux, B.M.; Grange, D.K.; Manwaring, L.; Tolmie, J.; Joss, S.; Study, D.D.D.; Cobben, J.M.; Duijkers, F.A.M.; Goehringer, J.M.; Challman, T.D.; Hennig, F.; Fischer, U.; Grimme, A.; Suckow, V.; Musante, L.; Nicholl, J.; Shaw, M.; Lodh, S.P.; Niu, Z.; Rosenfeld, J.A.; Stankiewicz, P.; Jentsch, T.J.; Gecz, J.; Field, M.; Kalscheuer, V.M.

Molecular Psychiatry 2018

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Ca2+-activated Cl−currents in the murine vomeronasal organ enhance neuronal spiking but are dispensable for male-male aggression

  • Jonas Münch; Gwendolyn Billig; Christian A. Huebner; Trese Leinders-Zufall; Frank Zufall; Thomas J. Jentsch

Journal of Biological Chemistry 2018

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LRRC8/VRAC anion channels are required for late stages of spermatid development in mice

  • Jennifer C. Lück; Dmytro Puchkov; Florian Ullrich; Thomas J. Jentsch

Journal of Biological Chemistry 2018

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LRRC8 N termini influence pore properties and gating of volume-regulated anion channels (VRACs)

  • Pingzheng Zhou; Maya M. Polovitskaya; Thomas J. Jentsch

Journal of Biological Chemistry 2018

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The ClC-K2 chloride channel is critical for salt handling in the distal nephron

  • Hennings, J.C.; Andrini, O.; Picard, N.; Paulais, M.; Huebner, A.K.; Cayuqueo, I.K.L.; Bignon, Y.; Keck, M.; Cornière, N.; Böhm, D.; Jentsch, T.J.; Chambrey, R.; Teulon, J.; Hübner, C.A.; Eladari, D.

Journal of the American Society of Nephrology 2017

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Loss of the Na+/H+ exchanger NHE8 causes male infertility in mice by disrupting acrosome formation

  • Karina Oberheide; Dmytro Puchkov; Thomas J. Jentsch

Journal of Biological Chemistry 2017

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Selective transport of neurotransmitters and –modulators by distinct volume-regulated LRRC8 anion channels

  • Darius Lutter; Florian Ullrich; Jennifer C. Lueck; Stefan Kempa; Thomas J. Jentsch

Journal of Cell Science 2017

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De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females.

  • Palmer EE; Stuhlmann T; Weinert S; Haan E; Van Esch H; Holvoet M; Boyle J; Leffler M; Raynaud M; Moraine C; van Bokhoven H; Kleefstra T; Kahrizi K; Najmabadi H; Ropers HH; Delgado MR; Sirsi D; Golla S; Sommer A; Pietryga MP
online lesen

Disruption of Kcc2-dependent inhibition of olfactory bulb output neurons suggests its importance in odour discrimination.

  • Gödde K; Gschwend O; Puchkov D; Pfeffer CK; Carleton A; Jentsch TJ
online lesen

The ClC-K2 Chloride Channel Is Critical for Salt Handling in the Distal Nephron.

  • Hennings JC; Andrini O; Picard N; Paulais M; Huebner AK; Cayuqueo IK; Bignon Y; Keck M; Cornière N; Böhm D; Jentsch TJ; Chambrey R; Teulon J; Hübner CA; Eladari D
online lesen

Inactivation and Anion Selectivity of Volume-Regulated VRAC Channels Depend on Carboxy-Terminal Residues of the First Extracellular Loop.

  • Ullrich F; Reincke SM; Voss FK; Stauber T; Jentsch TJ
online lesen

VRACs and other ion channels and transporters in the regulation of cell volume and beyond.

  • Jentsch TJ
online lesen

Inactivation and anion selectivity of volume-regulated anion channels (VRACs) depend on c-terminal residues of the first extracellular loop

  • Ullrich, F.; Reincke, S.M.; Voss, F.K.; Stauber, T.; Jentsch, T.J.

Journal of Biological Chemistry 2016

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KCNQ Potassium Channels Modulate Sensitivity of Skin Down-hair (D-hair) Mechanoreceptors.

  • Schütze S; Orozco IJ; Jentsch TJ
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VRAC: molecular identification as LRRC8 heteromers with differential functions.

  • Jentsch TJ; Lutter D; Planells-Cases R; Ullrich F; Voss FK
online lesen

X-exome sequencing of 405 unresolved families identifies seven novel intellectual disability genes.

  • Hu H; Haas SA; Chelly J; Van Esch H; Raynaud M; de Brouwer AP; Weinert S; Froyen G; Frints SG; Laumonnier F; Zemojtel T; Love MI; Richard H; Emde AK; Bienek M; Jensen C; Hambrock M; Fischer U; Langnick C; Feldkamp M
online lesen

In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11.

  • Varga RE; Khundadze M; Damme M; Nietzsche S; Hoffmann B; Stauber T; Koch N; Hennings JC; Franzka P; Huebner AK; Kessels MM; Biskup C; Jentsch TJ; Qualmann B; Braulke T; Kurth I; Beetz C; Hübner CA
online lesen

Optogenetic acidification of synaptic vesicles and lysosomes.

  • Rost BR; Schneider F; Grauel MK; Wozny C; Bentz CG; Blessing A; Rosenmund T; Jentsch TJ; Schmitz D; Hegemann P; Rosenmund C
online lesen

Subunit composition of VRAC channels determines substrate specificity and cellular resistance to Pt-based anti-cancer drugs.

  • Planells-Cases R; Lutter D; Guyader C; Gerhards NM; Ullrich F; Elger DA; Kucukosmanoglu A; Xu G; Voss FK; Reincke SM; Stauber T; Blomen VA; Vis DJ; Wessels LF; Brummelkamp TR; Borst P; Rottenberg S; Jentsch TJ
online lesen

Departure gate of acidic Ca²⁺ confirmed.

  • Jentsch TJ; Hoegg-Beiler MB; Vogt J
online lesen

Discovery of CLC transport proteins: cloning, structure, function and pathophysiology.

  • Jentsch TJ
online lesen

KCNQ5 K(+) channels control hippocampal synaptic inhibition and fast network oscillations.

  • Fidzinski P; Korotkova T; Heidenreich M; Maier N; Schuetze S; Kobler O; Zuschratter W; Schmitz D; Ponomarenko A; Jentsch TJ
online lesen

Discovery of CLC transport proteins: cloning, structure, function and pathophysiology.

  • Jentsch TJ
online lesen

Stretch-activation of angiotensin II type 1a receptors contributes to the myogenic response of mouse mesenteric and renal arteries.

  • Schleifenbaum J; Kassmann M; Szijártó IA; Hercule HC; Tano JY; Weinert S; Heidenreich M; Pathan AR; Anistan YM; Alenina N; Rusch NJ; Bader M; Jentsch TJ; Gollasch M
online lesen

Transport activity and presence of ClC-7/Ostm1 complex account for different cellular functions.

  • Weinert S; Jabs S; Hohensee S; Chan WL; Kornak U; Jentsch TJ
online lesen

Identification of LRRC8 heteromers as an essential component of the volume-regulated anion channel VRAC.

  • Voss FK; Ullrich F; Münch J; Lazarow K; Lutter D; Mah N; Andrade-Navarro MA; von Kries JP; Stauber T; Jentsch TJ
online lesen

CLCN7 and TCIRG1 mutations differentially affect bone matrix mineralization in osteopetrotic individuals.

  • Barvencik F; Kurth I; Koehne T; Stauber T; Zustin J; Tsiakas K; Ludwig CF; Beil FT; Pestka JM; Hahn M; Santer R; Supanchart C; Kornak U; Del Fattore A; Jentsch TJ; Teti A; Schulz A; Schinke T; Amling M
online lesen

Disrupting MLC1 and GlialCAM and ClC-2 interactions in leukodystrophy entails glial chloride channel dysfunction.

  • Hoegg-Beiler MB; Sirisi S; Orozco IJ; Ferrer I; Hohensee S; Auberson M; Gödde K; Vilches C; de Heredia ML; Nunes V; Estévez R; Jentsch TJ
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A missense mutation accelerating the gating of the lysosomal Cl-/H+-exchanger ClC-7/Ostm1 causes osteopetrosis with gingival hamartomas in cattle.

  • Sartelet A; Stauber T; Coppieters W; Ludwig CF; Fasquelle C; Druet T; Zhang Z; Ahariz N; Cambisano N; Jentsch TJ; Charlier C
online lesen

ClC-7 expression levels critically regulate bone turnover, but not gastric acid secretion.

  • Supanchart C; Wartosch L; Schlack C; Kühnisch J; Felsenberg D; Fuhrmann JC; de Vernejoul MC; Jentsch TJ; Kornak U
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From mice to man: chloride transport in leukoencephalopathy.

  • Jentsch TJ
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Common gating of both CLC transporter subunits underlies voltage-dependent activation of the 2Cl-/1H+ exchanger ClC-7/Ostm1.

  • Ludwig CF; Ullrich F; Leisle L; Stauber T; Jentsch TJ
online lesen

Exome sequencing reveals new causal mutations in children with epileptic encephalopathies.

  • Veeramah KR; Johnstone L; Karafet TM; Wolf D; Sprissler R; Salogiannis J; Barth-Maron A; Greenberg ME; Stuhlmann T; Weinert S; Jentsch TJ; Pazzi M; Restifo LL; Talwar D; Erickson RP; Hammer MF
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Vestibular role of KCNQ4 and KCNQ5 K+ channels revealed by mouse models.

  • Spitzmaul G; Tolosa L; Winkelman BH; Heidenreich M; Frens MA; Chabbert C; de Zeeuw CI; Jentsch TJ
online lesen

Chloride in vesicular trafficking and function.

  • Stauber T; Jentsch TJ
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A mouse model for distal renal tubular acidosis reveals a previously unrecognized role of the V-ATPase a4 subunit in the proximal tubule.

  • Hennings JC; Picard N; Huebner AK; Stauber T; Maier H; Brown D; Jentsch TJ; Vargas-Poussou R; Eladari D; Hübner CA
online lesen

Cell biology and physiology of CLC chloride channels and transporters.

  • Stauber T; Weinert S; Jentsch TJ
online lesen

GlialCAM, a protein defective in a leukodystrophy, serves as a ClC-2 Cl(-) channel auxiliary subunit.

  • Jeworutzki E; López-Hernández T; Capdevila-Nortes X; Sirisi S; Bengtsson L; Montolio M; Zifarelli G; Arnedo T; Müller CS; Schulte U; Nunes V; Martínez A; Jentsch TJ; Gasull X; Pusch M; Estévez R
online lesen

Raising cytosolic Cl- in cerebellar granule cells affects their excitability and vestibulo-ocular learning.

  • Seja P; Schonewille M; Spitzmaul G; Badura A; Klein I; Rudhard Y; Wisden W; Hübner CA; De Zeeuw CI; Jentsch TJ
online lesen

KCNQ4 K(+) channels tune mechanoreceptors for normal touch sensation in mouse and man.

  • Heidenreich M; Lechner SG; Vardanyan V; Wetzel C; Cremers CW; De Leenheer EM; Aránguez G; Moreno-Pelayo MÁ; Jentsch TJ; Lewin GR
online lesen

Enhanced Synaptic Activity and Epileptiform Events in the Embryonic KCC2 Deficient Hippocampus.

  • Khalilov I; Chazal G; Chudotvorova I; Pellegrino C; Corby S; Ferrand N; Gubkina O; Nardou R; Tyzio R; Yamamoto S; Jentsch TJ; Hübner CA; Gaiarsa JL; Ben-Ari Y; Medina I
online lesen

Generation and analyses of R8L barttin knockin mouse.

  • Nomura N; Tajima M; Sugawara N; Morimoto T; Kondo Y; Ohno M; Uchida K; Mutig K; Bachmann S; Soleimani M; Ohta E; Ohta A; Sohara E; Okado T; Rai T; Jentsch TJ; Sasaki S; Uchida S
online lesen

ClC-7 is a slowly voltage-gated 2Cl(-)/1H(+)-exchanger and requires Ostm1 for transport activity.

  • Leisle L; Ludwig CF; Wagner FA; Jentsch TJ; Stauber T
online lesen

Ca2+-activated Cl− currents are dispensable for olfaction.

  • Billig GM; Pál B; Fidzinski P; Jentsch TJ
online lesen

The CLC family of chloride channels and transporters

  • Stauber, T.; Novarino, G.; Jentsch, T.J.

Physiology and Pathology of chloride transporters and channels in the nervous system 2010

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Sorting motifs of the endosomal/lysosomal CLC chloride transporters.

  • Stauber T; Jentsch TJ
online lesen

A cation counterflux supports lysosomal acidification.

  • Steinberg BE; Huynh KK; Brodovitch A; Jabs S; Stauber T; Jentsch TJ; Grinstein S
online lesen

Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation.

  • Weinert S; Jabs S; Supanchart C; Schweizer M; Gimber N; Richter M; Rademann J; Stauber T; Kornak U; Jentsch TJ
online lesen

The KCNQ5 potassium channel mediates a component of the afterhyperpolarization current in mouse hippocampus.

  • Tzingounis AV; Heidenreich M; Kharkovets T; Spitzmaul G; Jensen HS; Nicoll RA; Jentsch TJ
online lesen

The late endosomal ClC-6 mediates proton/chloride countertransport in heterologous plasma membrane expression.

  • Neagoe I; Stauber T; Fidzinski P; Bergsdorf EY; Jentsch TJ
online lesen

Endosomal chloride-proton exchange rather than chloride conductance is crucial for renal endocytosis.

  • Novarino G; Weinert S; Rickheit G; Jentsch TJ
online lesen

Analysis of CLCN2 as candidate gene for megalencephalic leukoencephalopathy with subcortical cysts.

  • Scheper GC; van Berkel CG; Leisle L; de Groot KE; Errami A; Jentsch TJ; Van der Knaap MS
online lesen

Role of ClC-5 in renal endocytosis is unique among ClC exchangers and does not require PY-motif-dependent ubiquitylation.

  • Rickheit G; Wartosch L; Schaffer S; Stobrawa SM; Novarino G; Weinert S; Jentsch TJ
online lesen

Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport.

  • Preston P; Wartosch L; Günzel D; Fromm M; Kongsuphol P; Ousingsawat J; Kunzelmann K; Barhanin J; Warth R; Jentsch TJ
online lesen

No evidence for a role of CLCN2 variants in idiopathic generalized epilepsy.

  • Niemeyer MI; Cid LP; Sepúlveda FV; Blanz J; Auberson M; Jentsch TJ
online lesen

Distinct neuropathologic phenotypes after disrupting the chloride transport proteins ClC-6 or ClC-7/Ostm1.

  • Pressey SN; O'Donnell KJ; Stauber T; Fuhrmann JC; Tyynelä J; Jentsch TJ; Cooper JD
online lesen

ClC-3--a granular anion transporter involved in insulin secretion?

  • Jentsch TJ; Maritzen T; Keating DJ; Zdebik AA; Thévenod F
online lesen

Potassium ion movement in the inner ear: insights from genetic disease and mouse models.

  • Zdebik AA; Wangemann P; Jentsch TJ
online lesen

Residues important for nitrate/proton coupling in plant and mammalian CLC transporters.

  • Bergsdorf EY; Zdebik AA; Jentsch TJ
online lesen

Chloride channelopathies.

  • Planells-Cases R; Jentsch TJ
online lesen

NKCC1-dependent GABAergic excitation drives synaptic network maturation during early hippocampal development.

  • Pfeffer CK; Stein V; Keating DJ; Maier H; Rinke I; Rudhard Y; Hentschke M; Rune GM; Jentsch TJ; Hübner CA
online lesen

Erratum: Comment and reply on: CLC chloride channels and transporters: from genes to protein structure,pathology and physiology (Critical Reviews in Biochemistry and Molecular Biology (2009) 44: 4 (63-64)

  • Ueno, R.; Jentsch, T.J.

Critical Reviews in Biochemistry and Molecular Biology 2009

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To the editor

  • Jentsch, T.J.

Critical Reviews in Biochemistry and Molecular Biology 2009

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Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7.

  • Wartosch L; Fuhrmann JC; Schweizer M; Stauber T; Jentsch TJ
online lesen

Physiological roles of CLC Cl(-)/H (+) exchangers in renal proximal tubules.

  • Plans V; Rickheit G; Jentsch TJ
online lesen

CLC chloride channels and transporters: from genes to protein structure, pathology and physiology.

  • Jentsch TJ
online lesen

Determinants of anion-proton coupling in mammalian endosomal CLC proteins.

  • Zdebik AA; Zifarelli G; Bergsdorf EY; Soliani P; Scheel O; Jentsch TJ; Pusch M
online lesen

Endocochlear potential depends on Cl- channels: mechanism underlying deafness in Bartter syndrome IV.

  • Rickheit G; Maier H; Strenzke N; Andreescu CE; De Zeeuw CI; Muenscher A; Zdebik AA; Jentsch TJ
online lesen

Role of the vesicular chloride transporter ClC-3 in neuroendocrine tissue.

  • Maritzen T; Keating DJ; Neagoe I; Zdebik AA; Jentsch TJ
online lesen

Kv7 channels: interaction with dopaminergic and serotonergic neurotransmission in the CNS.

  • Hansen HH; Waroux O; Seutin V; Jentsch TJ; Aznar S; Mikkelsen JD
online lesen

Channelopathies of transepithelial transport and vesicular function.

  • Hübner CA; Jentsch TJ
online lesen

Disruption of erythroid K-Cl cotransporters alters erythrocyte volume and partially rescues erythrocyte dehydration in SAD mice.

  • Rust MB; Alper SL; Rudhard Y; Shmukler BE; Vicente R; Brugnara C; Trudel M; Jentsch TJ; Hübner CA
online lesen

Chloride and the endosomal-lysosomal pathway: emerging roles of CLC chloride transporters.

  • Jentsch TJ
online lesen

Detection and differentiation of sensorineural hearing loss in mice using auditory steady-state responses and transient auditory brainstem responses.

  • Pauli-Magnus D; Hoch G; Strenzke N; Anderson S; Jentsch TJ; Moser T
online lesen

Leukoencephalopathy upon disruption of the chloride channel ClC-2.

  • Blanz J; Schweizer M; Auberson M; Maier H; Muenscher A; Hübner CA; Jentsch TJ
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Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.

  • Poët M; Kornak U; Schweizer M; Zdebik AA; Scheel O; Hoelter S; Wurst W; Schmitt A; Fuhrmann JC; Planells-Cases R; Mole SE; Hübner CA; Jentsch TJ
online lesen

Kidney-specific upregulation of vitamin D3 target genes in ClC-5 KO mice.

  • Maritzen T; Rickheit G; Schmitt A; Jentsch TJ
online lesen

Structural determinants of M-type KCNQ (Kv7) K+ channel assembly.

  • Schwake M; Athanasiadu D; Beimgraben C; Blanz J; Beck C; Jentsch TJ; Saftig P; Friedrich T
online lesen

ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function.

  • Lange PF; Wartosch L; Jentsch TJ; Fuhrmann JC
online lesen

Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness.

  • Kharkovets T; Dedek K; Maier H; Schweizer M; Khimich D; Nouvian R; Vardanyan V; Leuwer R; Moser T; Jentsch TJ
online lesen

Neurogenic mechanisms contribute to hypertension in mice with disruption of the K-Cl cotransporter KCC3.

  • Rust MB; Faulhaber J; Budack MK; Pfeffer C; Maritzen T; Didié M; Beck FX; Boettger T; Schubert R; Ehmke H; Jentsch TJ; Hübner CA
online lesen

Mice with a targeted disruption of the Cl-/HCO3- exchanger AE3 display a reduced seizure threshold.

  • Hentschke M; Wiemann M; Hentschke S; Kurth I; Hermans-Borgmeyer I; Seidenbecher T; Jentsch TJ; Gal A; Hübner CA
online lesen

Physiological Functions of the CLC Chloride Transport Proteins

  • Maritzen, T.; Blanz, J.; Jentsch, T.

Advances in Molecular and Cell Biology 2006

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Chloride channel diseases resulting from impaired transepithelial transport or vesicular function.

  • Jentsch TJ; Maritzen T; Zdebik AA
online lesen

Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins.

  • Scheel O; Zdebik AA; Lourdel S; Jentsch TJ
online lesen

CLC chloride channels and transporters.

  • Jentsch TJ; Neagoe I; Scheel O
online lesen

Molecular determinants of KCNQ (Kv7) K+ channel sensitivity to the anticonvulsant retigabine.

  • Schenzer A; Friedrich T; Pusch M; Saftig P; Jentsch TJ; Grötzinger J; Schwake M
online lesen

Chloride transport in the kidney: lessons from human disease and knockout mice.

  • Jentsch TJ
online lesen

Unique structure and function of chloride transporting CLC proteins.

  • Pusch M; Jentsch TJ
online lesen

Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration.

  • Kasper D; Planells-Cases R; Fuhrmann JC; Scheel O; Zeitz O; Ruether K; Schmitt A; Poët M; Steinfeld R; Schweizer M; Kornak U; Jentsch TJ
online lesen

Physiological functions of CLC Cl- channels gleaned from human genetic disease and mouse models.

  • Jentsch TJ; Poët M; Fuhrmann JC; Zdebik AA
online lesen

Ion channels: function unravelled by dysfunction.

  • Jentsch TJ; Hübner CA; Fuhrmann JC
online lesen

Plasmodium induces swelling-activated ClC-2 anion channels in the host erythrocyte.

  • Huber SM; Duranton C; Henke G; Van De Sand C; Heussler V; Shumilina E; Sandu CD; Tanneur V; Brand V; Kasinathan RS; Lang KS; Kremsner PG; Hübner CA; Rust MB; Dedek K; Jentsch TJ; Lang F
online lesen

Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models.

  • Zdebik AA; Cuffe JE; Bertog M; Korbmacher C; Jentsch TJ
online lesen

Functional and structural conservation of CBS domains from CLC chloride channels.

  • Estévez R; Pusch M; Ferrer-Costa C; Orozco M; Jentsch TJ
online lesen

Expression of the KCl cotransporter KCC2 parallels neuronal maturation and the emergence of low intracellular chloride.

  • Stein V; Hermans-Borgmeyer I; Jentsch TJ; Hübner CA
online lesen

Conservation of chloride channel structure revealed by an inhibitor binding site in ClC-1.

  • Estévez R; Schroeder BC; Accardi A; Jentsch TJ; Pusch M
online lesen

A carboxy-terminal domain determines the subunit specificity of KCNQ K+ channel assembly.

  • Schwake M; Jentsch TJ; Friedrich T
online lesen

The ClC-5 chloride channel knock-out mouse - an animal model for Dent's disease.

  • Günther W; Piwon N; Jentsch TJ
online lesen

Erratum: Molecular structure and physiological function of chloride channels (Physiological Reviews (April 2002) 82 (503-568))

  • Jentsch, T.J.; Stein, V.; Weinreich, F.; Zdebik, A.A.

Physiological Reviews 2003

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The CIC-5 chloride channel knock-out mouse - An animal model for Dent's disease

  • Günther, W.; Piwon, N.; Jentsch, T.J.

Pflugers Archiv European Journal of Physiology 2003

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Loss of K-Cl co-transporter KCC3 causes deafness, neurodegeneration and reduced seizure threshold.

  • Boettger T; Rust MB; Maier H; Seidenbecher T; Schweizer M; Keating DJ; Faulhaber J; Ehmke H; Pfeffer C; Scheel O; Lemcke B; Horst J; Leuwer R; Pape HC; Völkl H; Hübner CA; Jentsch TJ
online lesen

Voltage-Gated Ion Channels

  • Christian A. H��bner; Thomas J. Jentsch

Wiley Encyclopedia of Molecular Medicine 2002

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Chloride channels are different.

  • Jentsch TJ
online lesen

Ion channel diseases.

  • Hübner CA; Jentsch TJ
online lesen

CLC chloride channels: correlating structure with function.

  • Estévez R; Jentsch TJ
online lesen

Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4.

  • Boettger T; Hübner CA; Maier H; Rust MB; Beck FX; Jentsch TJ
online lesen

Molecular structure and physiological function of chloride channels.

  • Jentsch TJ; Stein V; Weinreich F; Zdebik AA
online lesen

Ion channel diseases

  • Hübner, C.A.; Jentsch, T.J.

Human Molecular Genetics 2002

online lesen

Disruption of KCC2 reveals an essential role of K-Cl cotransport already in early synaptic inhibition.

  • Hübner CA; Stein V; Hermans-Borgmeyer I; Meyer T; Ballanyi K; Jentsch TJ
online lesen

Mutations in CAV3 cause mechanical hyperirritability of skeletal muscle in rippling muscle disease.

  • Betz RC; Schoser BG; Kasper D; Ricker K; Ramírez A; Stein V; Torbergsen T; Lee YA; Nöthen MM; Wienker TF; Malin JP; Propping P; Reis A; Mortier W; Jentsch TJ; Vorgerd M; Kubisch C
online lesen

Barttin is a Cl- channel beta-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion.

  • Estévez R; Boettger T; Stein V; Birkenhäger R; Otto E; Hildebrandt F; Jentsch TJ
online lesen

Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel.

  • Dedek K; Kunath B; Kananura C; Reuner U; Jentsch TJ; Steinlein OK
online lesen

Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption.

  • Bösl MR; Stein V; Hübner C; Zdebik AA; Jordt SE; Mukhopadhyay AK; Davidoff MS; Holstein AF; Jentsch TJ
online lesen

Tissue distribution and subcellular localization of the ClC-5 chloride channel in rat intestinal cells.

  • Vandewalle A; Cluzeaud F; Peng KC; Bens M; Lüchow A; Günther W; Jentsch TJ
online lesen

Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man.

  • Kornak U; Kasper D; Bösl MR; Kaiser E; Schweizer M; Schulz A; Friedrich W; Delling G; Jentsch TJ
online lesen

Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus.

  • Stobrawa SM; Breiderhoff T; Takamori S; Engel D; Schweizer M; Zdebik AA; Bösl MR; Ruether K; Jahn H; Draguhn A; Jahn R; Jentsch TJ
online lesen

Tissue distribution and subcellular localization of the C1C-5 chloride channel in rat intestinal cells

  • Vandewalle, A.; Cluzeaud, F.; Peng, K.-C.; Bens, M.; Lüchow, A.; Günther, W.; Jentsch, T.J.

American Journal of Physiology - Cell Physiology 2001

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Planned law could put German research at risk [4]

  • Jentsch, T.

Nature 2001

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An internalization signal in ClC-5, an endosomal Cl-channel mutated in dent's disease.

  • Schwake M; Friedrich T; Jentsch TJ
online lesen

Pores formed by single subunits in mixed dimers of different CLC chloride channels.

  • Weinreich F; Jentsch TJ
online lesen

A constitutively open potassium channel formed by KCNQ1 and KCNE3.

  • Schroeder BC; Waldegger S; Fehr S; Bleich M; Warth R; Greger R; Jentsch TJ
online lesen

Pathophysiology of KCNQ channels: neonatal epilepsy and progressive deafness.

  • Jentsch TJ; Schroeder BC; Kubisch C; Friedrich T; Stein V
online lesen

Mutations in the a3 subunit of the vacuolar H(+)-ATPase cause infantile malignant osteopetrosis.

  • Kornak U; Schulz A; Friedrich W; Uhlhaas S; Kremens B; Voit T; Hasan C; Bode U; Jentsch TJ; Kubisch C
online lesen

Functional and structural analysis of ClC-K chloride channels involved in renal disease.

  • Waldegger S; Jentsch TJ
online lesen

Inhibition of KCNQ1-4 potassium channels expressed in mammalian cells via M1 muscarinic acetylcholine receptors.

  • Selyanko AA; Hadley JK; Wood IC; Abogadie FC; Jentsch TJ; Brown DA
online lesen

ClC-5 Cl- -channel disruption impairs endocytosis in a mouse model for Dent's disease.

  • Piwon N; Günther W; Schwake M; Bösl MR; Jentsch TJ
online lesen

Neuronal KCNQ potassium channels: physiology and role in disease.

  • Jentsch TJ
online lesen

KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.

  • Kharkovets T; Hardelin JP; Safieddine S; Schweizer M; El-Amraoui A; Petit C; Jentsch TJ
online lesen

KCNQ5, a novel potassium channel broadly expressed in brain, mediates M-type currents.

  • Schroeder BC; Hechenberger M; Weinreich F; Kubisch C; Jentsch TJ
online lesen

Characterization of renal chloride channel (CLCN5) mutations in Dent's disease.

  • Yamamoto K; Cox JP; Friedrich T; Christie PT; Bald M; Houtman PN; Lapsley MJ; Patzer L; Tsimaratos M; Van'T Hoff WG; Yamaoka K; Jentsch TJ; Thakker RV
online lesen

From tonus to tonicity: physiology of CLC chloride channels.

  • Waldegger S; Jentsch TJ
online lesen

Neurological diseases caused by ion-channel mutations.

  • Weinreich F; Jentsch TJ
online lesen

Surface expression and single channel properties of KCNQ2/KCNQ3, M-type K+ channels involved in epilepsy.

  • Schwake M; Pusch M; Kharkovets T; Jentsch TJ
online lesen

Mutations in the a3 subunit of the vacuolar H<sup>+</sup>-ATPase cause infantile malignant osteopetrosis

  • Kornak, U.; Schulz, A.; Friedrich, W.; Uhlhaas, S.; Kremens, B.; Voit, T.; Hasan, C.; Bode, U.; Jentsch, T.J.; Kubisch, C.

Human Molecular Genetics 2000

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From tonus to tonicity: Physiology of CLC chloride channels

  • Waldegger, S.; Jentsch, T.J.

Journal of the American Society of Nephrology 2000

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Characterization of renal chloride channel (CLCN5) mutations in Dent's disease

  • Yamamoto, K.; Cox, J.P.D.T.; Friedrich, T.; Christie, P.T.; Bald, M.; Houtman, P.N.; Lapsley, M.J.; Patzer, L.; Tsimaratos, M.; Van't Hoff, W.G.; Yamaoka, K.; Jentsch, T.J.; Thakker, R.V.

Journal of the American Society of Nephrology 2000

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CLC chloride channels in Caenorhabditis elegans.

  • Schriever AM; Friedrich T; Pusch M; Jentsch TJ
online lesen

The CLC chloride channel family.

  • Jentsch TJ; Friedrich T; Schriever A; Yamada H
online lesen

Mutational analysis demonstrates that ClC-4 and ClC-5 directly mediate plasma membrane currents.

  • Friedrich T; Breiderhoff T; Jentsch TJ
online lesen

Chloride dependence of hyperpolarization-activated chloride channel gates.

  • Pusch M; Jordt SE; Stein V; Jentsch TJ
online lesen

KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness.

  • Kubisch C; Schroeder BC; Friedrich T; Lütjohann B; El-Amraoui A; Marlin S; Petit C; Jentsch TJ
online lesen

Moderate loss of function of cyclic-AMP-modulated KCNQ2/KCNQ3 K+ channels causes epilepsy.

  • Schroeder BC; Kubisch C; Stein V; Jentsch TJ
online lesen

Functional characterization of renal chloride channel, CLCN5, mutations associated with Dent'sJapan disease.

  • Igarashi T; Günther W; Sekine T; Inatomi J; Shiraga H; Takahashi S; Suzuki J; Tsuru N; Yanagihara T; Shimazu M; Jentsch TJ; Thakker RV
online lesen

ClC-1 chloride channel mutations in myotonia congenita: variable penetrance of mutations shifting the voltage dependence.

  • Kubisch C; Schmidt-Rose T; Fontaine B; Bretag AH; Jentsch TJ
online lesen

Golgi localization and functionally important domains in the NH2 and COOH terminus of the yeast CLC putative chloride channel Gef1p.

  • Schwappach B; Stobrawa S; Hechenberger M; Steinmeyer K; Jentsch TJ
online lesen

Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions.

  • Rychkov GY; Pusch M; Roberts ML; Jentsch TJ; Bretag AH
online lesen

Determinants of slow gating in ClC-0, the voltage-gated chloride channel of Torpedo marmorata.

  • Fong P; Rehfeldt A; Jentsch TJ
online lesen

Novel muscle chloride channel (CLCN1) mutations in myotonia congenita with various modes of inheritance including incomplete dominance and penetrance.

  • Plassart-Schiess E; Gervais A; Eymard B; Lagueny A; Pouget J; Warter JM; Fardeau M; Jentsch TJ; Fontaine B
online lesen

Molecular diagnosis of McArdle disease: revised genomic structure of the myophosphorylase gene and identification of a novel mutation.

  • Kubisch C; Wicklein EM; Jentsch TJ
online lesen

KCNQ2, the first gene found to be mutated in human generalized idiopathic epilepsy,Le KCNQ2 est le premier gene pour lequel une mutation est demontree chez des patients souffrant d'epilepsie generalisee idiopathique

  • Steinlein, O.K.; Jentsch, T.J.

Pathologie Biologie 1998

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Determinants of slow gating in ClC-0, the voltage-gated chloride channel of Torpedo marmorata

  • Fong, P.; Rehfeldt, A.; Jentsch, T.J.

American Journal of Physiology - Cell Physiology 1998

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Molecular physiology of renal chloride channels.

  • Steinmeyer K; Jentsch TJ
online lesen

ClC-5, the chloride channel mutated in Dent's disease, colocalizes with the proton pump in endocytotically active kidney cells.

  • Günther W; Lüchow A; Cluzeaud F; Vandewalle A; Jentsch TJ
online lesen

Characterization of the hyperpolarization-activated chloride current in dissociated rat sympathetic neurons.

  • Clark S; Jordt SE; Jentsch TJ; Mathie A
online lesen

A potassium channel mutation in neonatal human epilepsy.

  • Biervert C; Schroeder BC; Kubisch C; Berkovic SF; Propping P; Jentsch TJ; Steinlein OK
online lesen

CLC chloride channels in human disease

  • Jentsch, T.J.

FASEB Journal 1997

online lesen

Localization and induction by dehydration of ClC-K chloride channels in the rat kidney

  • Vandewalle, A.; Cluzeaud, F.; Bens, M.; Kieferle, S.; Steinmeyer, K.; Jentsch, T.J.

American Journal of Physiology - Renal Physiology 1997

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Molecular dissection of gating in the ClC-2 chloride channel.

  • Jordt SE; Jentsch TJ
online lesen

Pathophysiological mechanisms of dominant and recessive KVLQT1 K+ channel mutations found in inherited cardiac arrhythmias.

  • Wollnik B; Schroeder BC; Kubisch C; Esperer HD; Wieacker P; Jentsch TJ
online lesen

Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders.

  • Lloyd SE; Gunther W; Pearce SH; Thomson A; Bianchi ML; Bosio M; Craig IW; Fisher SE; Scheinman SJ; Wrong O; Jentsch TJ; Thakker RV
online lesen

Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1.

  • Schmidt-Rose T; Jentsch TJ
online lesen

Idiopathic low molecular weight proteinuria associated with hypercalciuric nephrocalcinosis in Japanese children is due to mutations of the renal chloride channel (CLCN5).

  • Lloyd SE; Pearce SH; Günther W; Kawaguchi H; Igarashi T; Jentsch TJ; Thakker RV
online lesen

Independent gating of single pores in CLC-0 chloride channels.

  • Ludewig U; Pusch M; Jentsch TJ
online lesen

Inward rectification in ClC-0 chloride channels caused by mutations in several protein regions.

  • Ludewig U; Jentsch TJ; Pusch M
online lesen

Transmembrane topology of a CLC chloride channel.

  • Schmidt-Rose T; Jentsch TJ
online lesen

Localization and induction by dehydration of ClC-K chloride channels in the rat kidney.

  • Vandewalle A; Cluzeaud F; Bens M; Kieferle S; Steinmeyer K; Jentsch TJ
online lesen

Analysis of a protein region involved in permeation and gating of the voltage-gated Torpedo chloride channel ClC-0.

  • Ludewig U; Jentsch TJ; Pusch M
online lesen

Chloride channels: an emerging molecular picture.

  • Jentsch TJ; Günther W
online lesen

Temperature dependence of fast and slow gating relaxations of ClC-0 chloride channels.

  • Pusch M; Ludewig U; Jentsch TJ
online lesen

Chloride channel myotonias

  • Steinmeyer, K.; Jentsch, T.J.

Neuroscientist 1996

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A family of putative chloride channels from Arabidopsis and functional complementation of a yeast strain with a CLC gene disruption.

  • Hechenberger M; Schwappach B; Fischer WN; Frommer WB; Jentsch TJ; Steinmeyer K
online lesen

Concentration and pH dependence of skeletal muscle chloride channel ClC-1.

  • Rychkov GY; Pusch M; Astill DS; Roberts ML; Jentsch TJ; Bretag AH
online lesen

Heteromultimeric CLC chloride channels with novel properties.

  • Lorenz C; Pusch M; Jentsch TJ
online lesen

Alteration of GABAA receptor function following gene transfer of the CLC-2 chloride channel.

  • Staley K; Smith R; Schaack J; Wilcox C; Jentsch TJ
online lesen

Two physically distinct pores in the dimeric ClC-0 chloride channel.

  • Ludewig U; Pusch M; Jentsch TJ
online lesen

Chloride channels: a molecular perspective.

  • Jentsch TJ
online lesen

A common molecular basis for three inherited kidney stone diseases.

  • Lloyd SE; Pearce SH; Fisher SE; Steinmeyer K; Schwappach B; Scheinman SJ; Harding B; Bolino A; Devoto M; Goodyer P; Rigden SP; Wrong O; Jentsch TJ; Craig IW; Thakker RV
online lesen

Cloning and functional expression of rat CLC-5, a chloride channel related to kidney disease.

  • Steinmeyer K; Schwappach B; Bens M; Vandewalle A; Jentsch TJ
online lesen

Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the CIC-1 chloride channel.

  • Pusch M; Steinmeyer K; Koch MC; Jentsch TJ
online lesen

Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCN1) leading to myotonia.

  • Meyer-Kleine C; Steinmeyer K; Ricker K; Jentsch TJ; Koch MC
online lesen

Molecular basis of epithelial Cl channels.

  • Fong P; Jentsch TJ
online lesen

Gating of the voltage-dependent chloride channel CIC-0 by the permeant anion.

  • Pusch M; Ludewig U; Rehfeldt A; Jentsch TJ
online lesen

Myotonias due to CLC-1 chloride channel mutations.

  • Jentsch TJ; Lorenz C; Pusch M; Steinmeyer K
online lesen

Properties of voltage-gated chloride channels of the ClC gene family.

  • Jentsch TJ; Günther W; Pusch M; Schwappach B
online lesen

Myotonias due to CLC-1 chloride channel mutations.

  • Jentsch, T.J.; Lorenz, C.; Pusch, M.; Steinmeyer, K.

Society of General Physiologists series 1995

online lesen

Myotonias due to CLC-1 chloride channel mutations

  • Jentsch, T.J.; Lorenz, C.; Pusch, M.; Steinmeyer, K.

Journal of General Physiology 1995

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Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCNI) leading to myotonia

  • Meyer-Kleine, C.; Steinmeyer, K.; Ricker, K.; Jentsch, T.J.; Koch, M.C.

American Journal of Human Genetics 1995

online lesen

Properties of voltage‐gated chloride channels of the ClC gene family.

  • Jentsch, T.J.; Günther, W.; Pusch, M.; Schwappach, B.

The Journal of Physiology 1995

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Chloride channel 2 gene (Clc2) maps to chromosome 16 of the mouse, extending a region of conserved synteny with human chromosome 3q

  • Lengeling, A.; Gronemeier, M.; Ronsiek, M.; Thiemann, A.; Jentsch, T.J.; Jockusch, H.

Genetical Research 1995

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Chloride channel 2 gene (Clc2) maps to chromosome 16 of the mouse, extending a region of conserved synteny with human chromosome 3q.

  • Lengeling A; Gronemeier M; Ronsiek M; Thiemann A; Jentsch TJ; Jockusch H
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ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family.

  • Brandt S; Jentsch TJ
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Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle.

  • Klocke R; Steinmeyer K; Jentsch TJ; Jockusch H
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Molecular physiology of voltage-gated chloride channels.

  • Pusch M; Jentsch TJ
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Molecular physiology of anion channels.

  • Jentsch TJ
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Two highly homologous members of the ClC chloride channel family in both rat and human kidney.

  • Kieferle S; Fong P; Bens M; Vandewalle A; Jentsch TJ
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Low single channel conductance of the major skeletal muscle chloride channel, ClC-1.

  • Pusch M; Steinmeyer K; Jentsch TJ
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Chapter 2 Molecular Biology of Voltage-Gated Chloride Channels

  • Thomas J. Jentsch

Chloride Channels 1994

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Structure and function of ClC chloride channels.

  • Jentsch, T.J.

The Japanese journal of physiology 1994

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Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle

  • Klocke, R.; Steinmeyer, K.; Jentsch, T.J.; Jockusch, H.

Journal of Biological Chemistry 1994

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Nonsense and missense mutations in the muscular chloride channel gene Clc- 1 of myotonic mice

  • Gronemeier, M.; Condie, A.; Prosser, J.; Steinmeyer, K.; Jentsch, T.J.; Jockusch, H.

Journal of Biological Chemistry 1994

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Multimeric structure of CIC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen)

  • Steinmeyer, K.; Lorenz, C.; Pusch, M.; Koch, M.C.; Jentsch, T.J.

EMBO Journal 1994

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Molecular physiology of voltage-gated chloride channels

  • Pusch, M.; Jentsch, T.J.

Physiological Reviews 1994

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Genomic organization of the human muscle chloride channel CIC-1 and analysis of novel mutations leading to Becker-type myotonia.

  • Lorenz C; Meyer-Kleine C; Steinmeyer K; Koch MC; Jentsch TJ
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Nonsense and missense mutations in the muscular chloride channel gene Clc-1 of myotonic mice.

  • Gronemeier M; Condie A; Prosser J; Steinmeyer K; Jentsch TJ; Jockusch H
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Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen).

  • Steinmeyer K; Lorenz C; Pusch M; Koch MC; Jentsch TJ
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Trinity of cation channels.

  • Jentsch TJ
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The ClC family of voltage-gated chloride channels: structure and function.

  • Jentsch TJ; Pusch M; Rehfeldt A; Steinmeyer K
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Evidence for genetic homogeneity in autosomal recessive generalised myotonia (Becker).

  • Koch MC; Ricker K; Otto M; Wolf F; Zoll B; Lorenz C; Steinmeyer K; Jentsch TJ
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Evidence for genetic homogeneity in autosomal recessive generalised myotonia (Becker)

  • Koch, M.C.; Ricker, K.; Otto, M.; Wolf, F.; Zoll, B.; Lorenz, C.; Steinmeyer, K.; Jentsch, T.J.

Journal of Medical Genetics 1993

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Recessive and dominant human myotonias are due to mutations of a chloride channel

  • Jentsch, T.J.

Biomedicine and Pharmacotherapy 1993

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Chloride channels.

  • Jentsch TJ
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Regions involved in the opening of CIC-2 chloride channel by voltage and cell volume.

  • Gründer S; Thiemann A; Pusch M; Jentsch TJ
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The skeletal muscle chloride channel in dominant and recessive human myotonia.

  • Koch MC; Steinmeyer K; Lorenz C; Ricker K; Wolf F; Otto M; Zoll B; Lehmann-Horn F; Grzeschik KH; Jentsch TJ
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A chloride channel widely expressed in epithelial and non-epithelial cells.

  • Thiemann A; Gründer S; Pusch M; Jentsch TJ
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Completely functional double-barreled chloride channel expressed from a single Torpedo cDNA.

  • Bauer CK; Steinmeyer K; Schwarz JR; Jentsch TJ
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Inactivation of muscle chloride channel by transposon insertion in myotonic mice.

  • Steinmeyer K; Klocke R; Ortland C; Gronemeier M; Jockusch H; Gründer S; Jentsch TJ
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Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel.

  • Steinmeyer K; Ortland C; Jentsch TJ
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Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes.

  • Jentsch TJ; Steinmeyer K; Schwarz G
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Cell culture of bovine corneal endothelial cells and its application to transport studies.

  • Wiederholt M; Jentsch TJ
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Primary structure of a novel 4-acetamido-4'-isothiocyanostilbene-2,2'-disulphonic acid (SITS)-binding membrane protein highly expressed in Torpedo californica electroplax.

  • Jentsch TJ; Garcia AM; Lodish HF
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Regulation of intracellular pH in cultured bovine retinal pigment epithelial cells.

  • Keller SK; Jentsch TJ; Janicke I; Wiederholt M
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Regulation of cytoplasmic pH of cultured bovine corneal endothelial cells in the absence and presence of bicarbonate.

  • Jentsch TJ; Korbmacher C; Janicke I; Fischer DG; Stahl F; Helbig H; Hollwede H; Cragoe EJ; Keller SK; Wiederholt M
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Kinetic properties of the sodium bicarbonate (carbonate) symport in monkey kidney epithelial cells (BSC-1). Interactions between Na+, HCO-3, and pH.

  • Jentsch TJ; Schwartz P; Schill BS; Langner B; Lepple AP; Keller SK; Wiederholt M
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Electrical properties of sodium bicarbonate symport in kidney epithelial cells (BSC-1).

  • Jentsch TJ; Matthes H; Keller SK; Wiederholt M
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The regulation of intracellular pH in monkey kidney epithelial cells (BSC-1). Roles of Na+/H+ antiport, Na+-HCO3(-)-(NaCO3-) symport, and Cl-/HCO3- exchange.

  • Jentsch TJ; Janicke I; Sorgenfrei D; Keller SK; Wiederholt M
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Interactions of pH and K+ conductance in cultured bovine retinal pigment epithelial cells.

  • Keller SK; Jentsch TJ; Koch M; Wiederholt M
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The regulation of intracellular pH in monkey kidney epithelial cells (BSC-1). Roles of Na<sup>+</sup>/H<sup>+</sup> antiport, Na<sup>+</sup>-HCO<inf>3</inf><sup>-</sup>-(NaCO<inf>3</inf><sup>-</sup>) symport, and Cl<sup>-</sup>/HCO<inf>3</inf><sup>-</sup> exchange

  • Jentsch, T.J.; Janicke, I.; Sorgenfrei, D.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1986

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Kinetic properties of the sodium bicarbonate (carbonate) symport in monkey kidney epithelial cells (BSC-1). Interactins between Na<sup>+</sup>, HCO<inf>3</inf><sup>-</sup>, and pH

  • Jentsch, T.J.; Schwartz, P.; Schill, B.S.; Langner, B.; Lepple, A.P.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1986

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Electrical properties of sodium bicarbonate symport in kidney epithelial cells (BSC-1)

  • Jentsch, T.J.; Matthes, H.; Keller, S.K.; Wiederholt, M.

American Journal of Physiology - Renal Fluid and Electrolyte Physiology 1986

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Interactions of pH and K<sup>+</sup> conductance in cultured bovine retinal pigment epithelial cells

  • Keller, S.K.; Jentsch, T.J.; Koch, M.; Wiederholt, M.

American Journal of Physiology - Cell Physiology 1986

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Kidney epithelial cells of monkey origin (BSC-1) express a sodium bicarbonate cotransport. Characterization by 22Na+ flux measurements.

  • Jentsch TJ; Schill BS; Schwartz P; Matthes H; Keller SK; Wiederholt M
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Observation of doubly charged triatomic cluster ions in field evaporation

  • Drachsel, W.; Jentsch, Th.; Gingerich, K.A.; Block, J.H.

Surface Science 1985

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Ion transport mechanisms in cultured bovine corneal endothelial cells

  • Jentsch, T.J.; Keller, S.K.; Wiederholt, M.

Current Eye Research 1985

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Response of the intracellular potentials of cultured bovine lens cells to ions and inhibitors.

  • Jentsch TJ; von der Haar B; Keller SK; Wiederholt M
online lesen

Ion transport mechanisms in cultured bovine corneal endothelial cells.

  • Jentsch TJ; Keller SK; Wiederholt M
online lesen

Anion dependence of electrical effects of bicarbonate and sodium on cultured bovine corneal endothelial cells.

  • Jentsch TJ; Matthes H; Keller SK; Wiederholt M
online lesen

Kidney epithelial cells of monkey origin (BSC-1) express a sodium bicarbonate cotransport. Characterization by <sup>22</sup>Na<sup>+</sup> flux measurements

  • Jentsch, T.J.; Schill, B.S.; Schwartz, P.; Matthes, H.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1985

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A bicarbonate-dependent process inhibitable by disulfonic stilbenes and a Na<sup>+</sup>/H<sup>+</sup> exchange mediate <sup>22</sup>Na<sup>+</sup> uptake into cultured bovine corneal endothelium

  • Jentsch, T.J.; Stahlknecht, T.R.; Hollwede, H.; Fischer, D.G.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1985

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Electrogenic sodium-bicarbonate symport in cultured corneal endothelial cells.

  • Wiederholt M; Jentsch TJ; Keller SK
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A bicarbonate-dependent process inhibitable by disulfonic stilbenes and a Na+/H+ exchange mediate 22Na+ uptake into cultured bovine corneal endothelium.

  • Jentsch TJ; Stahlknecht TR; Hollwede H; Fischer DG; Keller SK; Wiederholt M
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Effect of bicarbonate, pH, methazolamide and stilbenes on the intracellular potentials of cultured bovine corneal endothelial cells.

  • Jentsch TJ; Koch M; Bleckmann H; Wiederholt M
online lesen

The calcium antagonist nisoldipine stimulates the electrolyte transport of the isolated frog skin.

  • Wiederholt M; Keller S; Krolik A; Jentsch TJ
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Evidence for coupled transport of bicarbonate and sodium in cultured bovine corneal endothelial cells.

  • Jentsch TJ; Keller SK; Koch M; Wiederholt M
online lesen

New techniques in surface specific mass analysis by photon induced field desorption

  • Drachsel, W.; Jentsch, Th.; Block, J.H.

International Journal of Mass Spectrometry and Ion Physics 1983

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Stability of doubly charged homonuclear trimeric metal clusters

  • Jentsch, Th.; Drachsel, W.; Block, J.H.

Chemical Physics Letters 1982

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Copper cluster ions in photon-induced field ionization mass spectra

  • Jentsch, Th.; Drachsel, W.; Block, J.H.

International Journal of Mass Spectrometry and Ion Physics 1981

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Signal shapes observed in photon-induced field ionization mass spectra

  • Block, J.H.; Jentsch, T.H.; Drachsel, W.

International Journal of Mass Spectrometry and Ion Physics 1981

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Post-field ionization of singly charged rhodium: An experimental and theoretical study

  • Ernst, N.; Jentsch, Th.

Physical Review B 1981

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Cl− Channels and Cl−/H+ Exchangers

  • Thomas J. Jentsch

Encyclopedia of Molecular Pharmacology

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