Portrait Thomas Jentsch

Prof. Dr. Dr.

Thomas Jentsch

Thomas J. Jentsch studied both medicine and physics at the FU Berlin. After having obtained doctoral degrees in both areas, he did postdoctoral research in transport physiology in Berlin and molecular cell biology with Harvey Lodish at the Whitehead Institute/MIT. In 1988, he was a founding member of the Center for Molecular Neurobiology Hamburg (ZMNH), where he made his first seminal discoveries. In 2006 he moved his lab to Berlin, joining the FMP and MDC. Thomas Jentsch’s research is concerned with ion transport in the broadest sense. He is arguably the world leader in the field of chloride transport.

'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 identities 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, infertility, renal salt loss and aldosteronism.

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.’

Curriculum Vitae

– 1991

Habilitation in Cell Biochemistry

University Medical Center Hamburg

1986 – 1988

1988 – 1993

1993 – 2006

since 2006

since 2006

Head of Department Physiology & Pathology of Ion Transport

FMP & MDC Berlin

Awards

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
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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
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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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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
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Ion transport mechanisms in cultured bovine corneal endothelial cells.

  • Jentsch TJ; Keller SK; Wiederholt M
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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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