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2018

LRRC8 amino-termini influence pore properties and gating of volume-regulated VRAC anion channels.
Zhou, P. and Polovitskaya, M.M. and Jentsch, T.J.
Journal of Biological Chemistry 293 (35): 13440-13451. 31 August 2018

LRRC8/VRAC anion channels are required for late stages of spermatid development in mice.
Lück, J.C. and Puchkov, D. and Ullrich, F. and Jentsch, T.J.
Journal of Biological Chemistry 293 (30): 11796-11808. 27 July 2018

CLC chloride channels and transporters: structure, function, physiology, and disease.
Jentsch, T.J. and Pusch, M.
Physiological Reviews 98 (3): 1493-1590. July 2018

Ca(2+)-activated Cl(-) currents in the murine vomeronasal organ enhance neuronal spiking but are dispensable for male-male aggression.
Münch, J. and Billig, G. and Huebner, C.A. and Leinders-Zufall, T. and Zufall, F. and Jentsch, T.J.
Journal of Biological Chemistry 293 (26): 10392-10403. 29 June 2018

Intestinal electrolyte and fluid secretion: a model in trouble?
Cid, L.P. and Jentsch, T.J. and Sepúlveda, F.V.
Journal of Physiology 596 (12): 2465-2466. 15 June 2018

LRRC8/VRAC anion channels enhance β-cell glucose sensing and insulin secretion.
Stuhlmann, T. and Planells-Cases, R. and Jentsch, T.J.
Nature Communications 9 (1): 1974. 17 May 2018

A gain-of-function mutation in the CLCN2 chloride channel gene causes primary aldosteronism.
Fernandes-Rosa, F.L. and Daniil, G. and Orozco, I.J. and Göppner, C. and El Zein, R. and Jain, V. and Boulkroun, S. and Jeunemaitre, X. and Amar, L. and Lefebvre, H. and Schwarzmayr, T. and Strom, T.M. and Jentsch, T.J. and Zennaro, M.C.
Nature Genetics 50 (3): 355-361. March 2018

K(2P) TASK-2 and KCNQ1/KCNE3 K(+) channels are major players contributing to intestinal anion and fluid secretion.
Julio-Kalajzić, F. and Villanueva, S. and Burgos, J. and Ojeda, M. and Cid, L.P. and Jentsch, T.J. and Sepúlveda, F.V.
Journal of Physiology 596 (3): 393-407. 1 February 2018

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. and Stuhlmann, T. and Weinert, S. and Haan, E. and Van Esch, H. and Holvoet, M. and Boyle, J. and Leffler, M. and Raynaud, M. and Moraine, C. and van Bokhoven, H. and Kleefstra, T. and Kahrizi, K. and Najmabadi, H. and Ropers, H.H. and Delgado, M.R. and Sirsi, D. and Golla, S. and Sommer, A. and Pietryga, M.P. and Chung, W.K. and Wynn, J. and Rohena, L. and Bernardo, E. and Hamlin, D. and Faux, B.M. and Grange, D.K. and Manwaring, L. and Tolmie, J. and Joss, S. and Cobben, J.M. and Duijkers, F.A.M. and Goehringer, J.M. and Challman, T.D. and Hennig, F. and Fischer, U. and Grimme, A. and Suckow, V. and Musante, L. and Nicholl, J. and Shaw, M. and Lodh, S.P. and Niu, Z. and Rosenfeld, J.A. and Stankiewicz, P. and Jentsch, T.J. and Gecz, J. and Field, M. and Kalscheuer, V.M.
Molecular Psychiatry 23 (2): 222-230. February 2018

2017

Loss of the Na(+)/H(+) exchanger NHE8 causes male infertility in mice by disrupting acrosome formation.
Oberheide, K. and Puchkov, D. and Jentsch, T.J.
Journal of Biological Chemistry 292 (26): 10845-10854. 30 June 2017

Disruption of the vacuolar-type H(+)-ATPase complex in liver causes MTORC1-independent accumulation of autophagic vacuoles and lysosomes.
Kissing, S. and Rudnik, S. and Damme, M. and Luellmann-Rauch, R. and Ichihara, A. and Kornak, U. and Eskelinen, E.L. and Jabs, S. and Heeren, J. and De Brabander, J.K. and Haas, A. and Saftig, P.
Autophagy 13 (4): 670-685. April 2017

Selective transport of neurotransmitters and modulators by distinct volume-regulated LRRC8 anion channels.
Lutter, D. and Ullrich, F. and Lueck, J.C. and Kempa, S. and Jentsch, T.J.
Journal of Cell Science 130 (6): 1122-1133. 15 March 2017

The ClC-K2 chloride channel is critical for salt handling in the distal nephron.
Hennings, J.C. and Andrini, O. and Picard, N. and Paulais, M. and Huebner, A.K. and Cayuqueo, I.K.L. and Bignon, Y. and Keck, M. and Cornière, N. and Böhm, D. and Jentsch, T.J. and Chambrey, R. and Teulon, J. and Hübner, C.A. and Eladari, D.
Journal of the American Society of Nephrology 28 (1): 209-217. January 2017

2016

Inactivation and anion selectivity of volume-regulated VRAC channels depend on carboxy-terminal residues of the first extracellular loop.
Ullrich, F. and Reincke, S.M. and Voss, F.K. and Stauber, T. and Jentsch, T.J.
Journal of Biological Chemistry 291 (33): 17040-17048. 12 August 2016

Disruption of Kcc2-dependent inhibition of olfactory bulb output neurons suggests its importance in odour discrimination.
Gödde, K. and Gschwend, O. and Puchkov, D. and Pfeffer, C.K. and Carleton, A. and Jentsch, T.J.
Nature Communications 7 : 12043. 8 July 2016

Lysosomal dysfunction caused by cellular accumulation of silica nanoparticles.
Schuetz, I. and Lopez-Hernandez, T. and Gao, Q. and Puchkov, D. and Jabs, S. and Nordmeyer, D. and Schmudde, M. and Ruehl, E. and Graf, C.M. and Haucke, V.
Journal of Biological Chemistry 291 (27): 14170-14184. 1 July 2016

VRACs and other ion channels and transporters in the regulation of cell volume and beyond.
Jentsch, T.J.
Nature Reviews Molecular Cell Biology 17 (5): 293-307. May 2016

KCNQ potassium channels modulate sensitivity of skin D-hair mechanoreceptors.
Schütze, S. and Orozco, I.J. and Jentsch, T.J.
Journal of Biological Chemistry 291 (11): 5566-5575. 11 March 2016

VRAC: molecular identification as LRRC8 heteromers with differential functions.
Jentsch, T.J. and Lutter, D. and Planells-Cases, R. and Ullrich, F. and Voss, F.K.
Pflugers Archiv 468 (3): 385-393. March 2016

X-exome sequencing of 405 unresolved families identifies seven novel intellectual disability genes.
Hu, H. and Haas, S.A. and Chelly, J. and Van Esch, H. and Raynaud, M. and de Brouwer, A.P.M. and Weinert, S. and Froyen, G. and Frints, S.G.M. and Laumonnier, F. and Zemojtel, T. and Love, M.I. and Richard, H. and Emde, A.K. and Bienek, M. and Jensen, C. and Hambrock, M. and Fischer, U. and Langnick, C. and Feldkamp, M. and Wissink-Lindhout, W. and Lebrun, N. and Castelnau, L. and Rucci, J. and Montjean, R. and Dorseuil, O. and Billuart, P. and Stuhlmann, T. and Shaw, M. and Corbett, M.A. and Gardner, A. and Willis-Owen, S. and Tan, C. and Friend, K.L. and Belet, S. and van Roozendaal, K.E.P. and Jimenez-Pocquet, M. and Moizard, M.P. and Ronce, N. and Sun, R. and O'Keeffe, S. and Chenna, R. and van Bömmel, A. and Goeke, J. and Hackett, A. and Field, M. and Christie, L. and Boyle, J. and Haan, E. and Nelson, J. and Turner, G. and Baynam, G. and Gillessen-Kaesbach, G. and Mueller, U. and Steinberger, D. and Budny, B. and Badura-Stronka, M. and Latos-Bieleńska, A. and Ousager, L.B. and Wieacker, P. and Rodríguez Criado, G. and Bondeson, M.L. and Annerén, G. and Dufke, A. and Cohen, M. and Van Maldergem, L. and Vincent-Delorme, C. and Echenne, B. and Simon-Bouy, B. and Kleefstra, T. and Willemsen, M. and Fryns, J.P. and Devriendt, K. and Ullmann, R. and Vingron, M. and Wrogemann, K. and Wienker, T.F. and Tzschach, A. and van Bokhoven, H. and Gecz, J. and Jentsch, T.J. and Chen, W. and Ropers, H.H. and Kalscheuer, V.M.
Molecular Psychiatry 21 (1): 133-148. January 2016

2015

Optogenetic acidification of synaptic vesicles and lysosomes.
Rost, B.R. and Schneider, F. and Grauel, M.K. and Wozny, C. and Bentz, C.G. and Blessing, A. and Rosenmund, T. and Jentsch, T.J. and Schmitz, D. and Hegemann, P. and Rosenmund, C.
Nature Neuroscience 18 (12): 1845-1852. December 2015

Subunit composition of VRAC channels determines substrate specificity and cellular resistance to Pt-based anti-cancer drugs.
Planells-Cases, R. and Lutter, D. and Guyader, C. and Gerhards, N.M. and Ullrich, F. and Elger, D.A. and Kucukosmanoglu, A. and Xu, G. and Voss, F.K. and Reincke, S.M. and Stauber, T. and Blomen, V.A. and Vis, D.J. and Wessels, L.F. and Brummelkamp, T.R. and Borst, P. and Rottenberg, S. and Jentsch, T.J.
EMBO Journal 34 (24): 2993-3008. December 2015

Discovery of CLC transport proteins: cloning, structure, function and pathophysiology.
Jentsch, T.J.
Journal of Physiology 593 (18): 4091-4109. 15 September 2015

In vivo evidence for lysosome depletion and impaired autophagic clearance in hereditary spastic paraplegia type SPG11.
Varga, R.E. and Khundadze, M. and Damme, M. and Nietzsche, S. and Hoffmann, B. and Stauber, T. and Koch, N. and Hennings, J.C. and Franzka, P. and Huebner, A.K. and Kessels, M.M. and Biskup, C. and Jentsch, T.J. and Qualmann, B. and Braulke, T. and Kurth, I. and Beetz, C. and Hübner, C.A.
PLoS Genetics 11 (8): e1005454. 18 August 2015

Departure gate of acidic Ca(2+) confirmed.
Jentsch, T.J. and Hoegg-Beiler, M.B. and Vogt, J.
EMBO Journal 34 (13): 1737-1739. 2 July 2015

KCNQ5 K(+) channels control hippocampal synaptic inhibition and fast network oscillations.
Fidzinski, P. and Korotkova, T. and Heidenreich, M. and Maier, N. and Schuetze, S. and Kobler, O. and Zuschratter, W. and Schmitz, D. and Ponomarenko, A. and Jentsch, T.J.
Nature Communications 6 : 6254. 4 February 2015

2014

Stretch-activation of angiotensin II type 1a receptors contributes to the myogenic response of mouse mesenteric and renal arteries.
Schleifenbaum, J. and Kassmann, M. and Szijártó, I.A. and Hercule, H.C. and Tano, J.Y. and Weinert, S. and Heidenreich, M. and Pathan, A.R. and Anistan, Y.M. and Alenina, N. and Rusch, N.J. and Bader, M. and Jentsch, T.J. and Gollasch, M.
Circulation Research 115 (2): 263-272. 7 July 2014

Transport activity and presence of ClC-7/Ostm1 complex account for different cellular functions.
Weinert, S. and Jabs, S. and Hohensee, S. and Chan, W.L. and Kornak, U. and Jentsch, T.J.
EMBO Reports 15 (7): 784-791. 1 July 2014

Identification of LRRC8 heteromers as an essential component of the volume-regulated anion channel VRAC.
Voss, F.K. and Ullrich, F. and Muench, J. and Lazarow, K. and Lutter, D. and Mah, N. and Andrade-Navarro, M.A. and von Kries, J.P. and Stauber, T. and Jentsch, T.J.
Science 344 (6184): 634-638. 9 May 2014

CLCN7 and TCIRG1 mutations differentially affect bone matrix mineralization in osteopetrotic individuals.
Barvencik, F. and Kurth, I. and Koehne, T. and Stauber, T. and Zustin, J. and Tsiakas, K. and Ludwig, C.F. and Beil, F.T. and Pestka, J.M. and Hahn, M. and Santer, R. and Supanchart, C. and Kornak, U. and Del Fattore, A. and Jentsch, T. and Teti, A. and Schulz, A. and Schinke, T. and Amling, M.
Journal of Bone and Mineral Research 29 (4): 982-991. April 2014

Disrupting MLC1 and GlialCAM and ClC-2 interactions in leukodystrophy entails glial chloride channel dysfunction.
Hoegg-Beiler, M.B. and Sirisi, S. and Orozco, I.J. and Ferrer, I. and Hohensee, S. and Auberson, M. and Goedde, K. and Vilches, C. and de Heredia, M.L. and Nunes, V. and Estévez, R. and Jentsch, T.J.
Nature Communications 5 : 3475. 19 March 2014

A missense mutation accelerating the gating of the lysosomal Cl(-)/H(+)-exchanger ClC-7/Ostm1 causes osteopetrosis with gingival hamartomas in cattle.
Sartelet, A. and Stauber, T. and Coppieters, W. and Ludwig, C.F. and Fasquelle, C. and Druet, T. and Zhang, Z. and Ahariz, N. and Cambisano, N. and Jentsch, T.J. and Charlier, C.
Disease Models & Mechanisms 7 (1): 119-128. January 2014

ClC-7 expression levels critically regulate bone turnover, but not gastric acid secretion.
Supanchart, C. and Wartosch, L. and Schlack, C. and Kühnisch, J. and Giehl, M. and Felsenberg, D. and Fuhrmann, J.C. and de Vernejoul, M.C. and Jentsch, T.J. and Kornak, U.
Bone 58 : 92-102. January 2014

2013

Common gating of both CLC subunits underlies voltage-dependent activation of the 2Cl(-)/1H(+) exchanger ClC-7/Ostm1.
Ludwig, C.F. and Ullrich, F. and Leisle, L. and Stauber, T. and Jentsch, T.J.
Journal of Biological Chemistry 288 (40): 28611-28619. 4 October 2013

Exome sequencing reveals new causal mutations in children with epileptic encephalopathies.
Veeramah, K.R. and Johnstone, L. and Karafet, T.M. and Wolf, D. and Sprissler, R. and Salogiannis, J. and Barth-Maron, A. and Greenberg, M.E. and Stuhlmann, T. and Weinert, S. and Jentsch, T.J. and Pazzi, M. and Restifo, L.L. and Talwar, D. and Erickson, R.P. and Hammer, M.F.
Epilepsia 54 (7): 1270-1281. July 2013

From mice to man: chloride transport in leukoencephalopathy.
Jentsch, T.J.
Lancet Neurology 12 (7): 626-628. July 2013

Potentiation of the transient receptor potential vanilloid 1 channel contributes to pruritogenesis in a rat model of liver disease.
Belghiti, M. and Estevez-Herrera, J. and Gimenez-Garzo, C. and Gonzalez-Usano, A. and Montoliu, C. and Ferrer-Montiel, A. and Felipo, V. and Planells-Cases, R.
Journal of Biological Chemistry 288 (14): 9675-9685. 5 April 2013

Vestibular role of KCNQ4 and KCNQ5 K(+) channels revealed by mouse models.
Spitzmaul, G. and Tolosa, L. and Winkelman, B.H.J. and Heidenreich, M. and Frens, M.A. and Chabbert, C. and de Zeeuw, C.I. and Jentsch, T.J.
Journal of Biological Chemistry 288 (13): 9334-9344. 29 March 2013

The new KCNQ2 activator 4-Chlor-N-(6-chlor-pyridin-3-yl)-benzamid displays anticonvulsant potential.
Boehlen, A. and Schwake, M. and Dost, R. and Kunert, A. and Fidzinski, P. and Heinemann, U. and Gebhardt, C.
British Journal of Pharmacology 168 (5): 1182-1200. March 2013

Chloride in vesicular trafficking and function.
Stauber, T. and Jentsch, T.J.
Annual Review of Physiology 75 : 453-477. 10 February 2013

2012

A mouse model for distal renal tubular acidosis reveals a previously unrecognized role of the V-ATPase a4 subunit in the proximal tubule.
Hennings, J.C. and Picard, N. and Huebner, A.K. and Stauber, T. and Maier, H. and Brown, D. and Jentsch, T.J. and Vargas-Poussou, R. and Eladari, D. and Huebner, C.A.
EMBO Molecular Medicine 4 (10): 1057-1071. October 2012

Cell biology and physiology of CLC chloride channels and transporters.
Stauber, T. and Weinert, S. and Jentsch, T.J.
Comprehensive Physiology 2 (3): 1701-1744. 1 July 2012

Elucidating membrane structure and protein behavior using giant plasma membrane vesicles.
Sezgin, E. and Kaiser, H.J. and Baumgart, T. and Schwille, P. and Simons, K. and Levental, I.
Nature Protocols 7 (6): 1042-1051. June 2012

GlialCAM, a protein defective in a leukodystrophy, serves as a ClC-2 Cl(-) channel auxiliary subunit.
Jeworutzki, E. and Lopez-Hernandez, T. and Capdevila-Nortes, X. and Sirisi, S. and Bengtsson, L. and Montolio, M. and Zifarelli, G. and Arnedo, T. and Mueller, C.S. and Schulte, U. and Nunes, V. and Martinez, A. and Jentsch, T.J. and Gasull, X. and Pusch, M. and Estevez, R.
Neuron 73 (5): 951-961. 8 March 2012

Raising cytosolic Cl(-) in cerebellar granule cells affects their excitability and vestibulo-ocular learning.
Seja, P. and Schonewille, M. and Spitzmaul, G. and Badura, A. and Klein, I. and Rudhard, Y. and Wisden, W. and Huebner, C.A. and De Zeeuw, C.I. and Jentsch, T.J.
EMBO Journal 31 (5): 1217-1230. 17 January 2012

KCNQ4 K(+) channels tune mechanoreceptors for normal touch sensation in mouse and man.
Heidenreich, M. and Lechner, S.G. and Vardanyan, V. and Wetzel, C. and Cremers, C.W. and De Leenheer, E.M. and Aranguez, G. and Moreno-Pelayo, M.A. and Jentsch, T.J. and Lewin, G.R.
Nature Neuroscience 15 (1): 138-145. 2012

2011

Enhanced synaptic activity and epileptiform events in the embryonic KCC2 deficient hippocampus.
Khalilov, I. and Chazal, G. and Chudotvorova, I. and Pellegrino, C. and Corby, S. and Ferrand, N. and Gubkina, O. and Nardou, R. and Tyzio, R. and Yamamoto, S. and Jentsch, T.J. and Huebner, C.A. and Gaiarsa, J.L. and Ben-Ari, Y. and Medina, I.
Frontiers in Cellular Neuroscience 5 : 23. 1 November 2011

Generation and analyses of R8L barttin knockin mouse.
Nomura, N. and Tajima, M. and Sugawara, N. and Morimoto, T. and Kondo, Y. and Ohno, M. and Uchida, K. and Mutig, K. and Bachmann, S. and Soleimani, M. and Ohta, E. and Ohta, A. and Sohara, E. and Okado, T. and Rai, T. and Jentsch, T.J. and Sasaki, S. and Uchida, S.
American Journal of Physiology Renal Physiology 301 (2): F297-F307. August 2011

ClC-7 is a slowly voltage-gated 2Cl(-)/1H(+)-exchanger and requires Ostm1 for transport activity.
Leisle, L. and Ludwig, C.F. and Wagner, F.A. and Jentsch, T.J. and Stauber, T.
EMBO Journal 30 (11): 2140-2152. 1 June 2011

Ca(2+)-activated Cl(-) currents are dispensable for olfaction.
Billig, G.M. and Pal, B. and Fidzinski, P. and Jentsch, T.J.
Nature Neuroscience 14 (6): 763-769. June 2011

Recurrent stroke due to a novel voltage sensor mutation in Ca(v)2.1 responds to verapamil.
Knierim, E. and Leisle, L. and Wagner, C. and Weschke, B. and Lucke, B. and Bohner, G. and Dreier, J.P. and Schuelke, M.
Stroke 42 (2): e14-e17. February 2011

2010

Distinct neuropathologic phenotypes after disrupting the chloride transport proteins ClC-6 or ClC-7/Ostm1.
Pressey, S.N. and O'Donnell, K.J. and Stauber, T. and Fuhrmann, J.C. and Tyynelae, J. and Jentsch, T.J. and Cooper, J.D.
Journal of Neuropathology and Experimental Neurology 69 (12): 1228-1246. December 2010

Sorting motifs of the endosomal/lysosomal CLC chloride transporters.
Stauber, T. and Jentsch, T.J.
Journal of Biological Chemistry 285 (45): 34537-34548. 5 November 2010

ClC-3-A granular anion transporter involved in insulin secretion?
Jentsch, T.J. and Maritzen, T. and Keating, D.J. and Zdebik, A.A. and Thevenod, F.
Cell Metabolism 12 (4): 307-308. 6 October 2010

The G215R mutation in the Cl-/H+-antiporter ClC-7 found in ADO II osteopetrosis does not abolish function but causes a severe trafficking defect.
Schulze, K. and Werner, J. and Stauber, T. and Henriksen, K. and Fendler, K.
PLoS ONE 5 (9): e12585. 7 September 2010

The late endosomal CLC-6 mediates proton/chloride countertransport in heterologous plasma membrane expression.
Neagoe, I. and Stauber, T. and Fidzinski, P. and Bergsdorf, E.Y. and Jentsch, T.J.
Journal of Biological Chemistry 285 (28): 21689-21697. 9 July 2010

A cation counterflux supports lysosomal acidification.
Steinberg, B.E. and Huynh, K.K. and Brodovitch, A. and Jabs, S. and Stauber, T. and Jentsch, T.J. and Grinstein, S.
Journal of Cell Biology 189 (7): 1171-1186. 28 June 2010

Endosomal chloride-proton exchange rather than chloride conductance is crucial for renal endocytosis.
Novarino, G. and Weinert, S. and Rickheit, G. and Jentsch, T.J.
Science 328 (5984): 1398-1401. 11 June 2010

Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation.
Weinert, S. and Jabs, S. and Supanchart, C. and Schweizer, M. and Gimber, N. and Richter, M. and Rademann, J. and Stauber, T. and Kornak, U. and Jentsch, T.J.
Science 328 (5984): 1401-1403. 11 June 2010

Role of CLC-5 in renal endocytosis is unique among CLC exchangers and does not require py-motif-dependent ubiquitylation.
Rickheit, G. and Wartosch, L. and Schaffer, S. and Stobrawa, S. and Novarino, G. and Weinert, S. and Jentsch, T.J.
Journal of Biological Chemistry 285 (23): 17595-17603. 4 June 2010

The KCNQ5 potassium channel mediates a component of the afterhyperpolarization current in mouse hippocampus.
Tzingounis, A.V. and Heidenreich, M. and Kharkovets, T. and Spitzmaul, G. and Jensen, H.S. and Nicoll, R.A. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 107 (22): 10232-10237. 1 June 2010

Analysis of CLCN2 as candidate gene for megalencephalic leukoencephalopathy with subcortical cysts.
Scheper, G.C. and van Berkel, C.G. and Leisle, L. and de Groot, K.E. and Errami, A. and Jentsch, T.J. and Van der Knaap, M.S.
Genetic Testing and Molecular Biomarkers 14 (2): 255-257. April 2010

Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport.
Preston, P. and Wartosch, L. and Guenzel, D. and Fromm, M. and Kongsuphol, P. and Ousingsawat, J. and Kunzelmann, K. and Barhanin, J. and Warth, R. and Jentsch, T.J.
Journal of Biological Chemistry 285 (10): 7165-7175. 5 March 2010

A role for chloride transport in lysosomal protein degradation.
Wartosch, L. and Stauber, T.
Autophagy 6 (1): 158-159. January 2010

No evidence for a role of CLCN2 variants in idiopathic generalized epilepsy.
Niemeyer, M.I. and Cid, L.P. and Sepulveda, F.V. and Blanz, J. and Auberson, M. and Jentsch, T.J.
Nature Genetics 42 (1): 3. January 2010

The CLC family of chloride channels and transporters.
Stauber, T. and Novarino, G. and Jentsch, T.J.
In: Physiology and Pathology of Chloride Transporters and Channels in the Nervous System : From Molecules to Diseases. Elsevier, 209-231. ISBN 978-0-12-374373-2 2010

2009

Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7.
Wartosch, L. and Fuhrmann, J.C. and Schweizer, M. and Stauber, T. and Jentsch, T.J.
FASEB Journal 23 (12): 4056-4068. December 2009

Potassium ion movement in the inner ear: insights from genetic disease and mouse models.
Zdebik, A.A. and Wangemann, P. and Jentsch, T.J.
Physiology 24 (5): 307-316. October 2009

Physiological roles of CLC Cl(-)/H (+) exchangers in renal proximal tubules.
Plans, V. and Rickheit, G. and Jentsch, T.J.
Pfluegers Archiv European Journal of Physiology 458 (1): 23-37. May 2009

Residues important for nitrate/proton coupling in plant and mammalian CLC transporters.
Bergsdorf, E.Y. and Zdebik, A.A. and Jentsch, T.J.
Journal of Biological Chemistry 284 (17): 11184-11193. 24 April 2009

NKCC1-dependent GABAergic excitation drives synaptic network maturation during early hippocampal development.
Pfeffer, C.K. and Stein, V. and Keating, D.J. and Maier, H. and Rinke, I. and Rudhard, Y. and Hentschke, M. and Rune, G.M. and Jentsch, T.J. and Huebner, C.A.
Journal of Neuroscience 29 (11): 3419-3430. 18 March 2009

Chloride channelopathies.
Planells-Cases, R. and Jentsch, T.J.
Biochimica et Biophysica Acta - Molecular Basis of Disease 1792 (3): 173-189. March 2009

Muscarinic acetylcholine receptors and voltage-gated calcium channels contribute to bidirectional synaptic plasticity at CA1-subiculum synapses.
Shor, O.L. and Fidzinski, P. and Behr, J.
Neuroscience Letters 449 (3): 220-223. 16 January 2009

2008

ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+ signals across the inner ear.
Anselmi, F. and Hernandez, V.H. and Crispino, G. and Seydel, A. and Ortolano, S. and Roper, S.D. and Kessaris, N. and Richardson, W. and Rickheit, G. and Filippov, M.A. and Monyer, H. and Mammano, F.
Proceedings of the National Academy of Sciences of the United States of America 105 (48): 18770-18775. 2 December 2008

Endocochlear potential depends on Cl(-) channels: mechanism underlying deafness in Bartter syndrome IV.
Rickheit, G. and Maier, H. and Strenzke, N. and Andreescu, C.E. and De Zeeuw, C.I. and Muenscher, A. and Zdebik, A.A. and Jentsch, T.J.
EMBO Journal 27 (21): 2907-2917. 5 November 2008

Role of the vesicular chloride transporter ClC-3 in neuroendocrine tissue.
Maritzen, T. and Keating, D.J. and Neagoe, I. and Zdebik, A.A. and Jentsch, T.J.
Journal of Neuroscience 28 (42): 10587-10598. 15 October 2008

Kv7 channels: interaction with dopaminergic and serotonergic neurotransmission in the CNS.
Hansen, H.H. and Waroux, O. and Seutin, V. and Jentsch, T.J. and Aznar, S. and Mikkelsen, J.D.
Journal of Physiology 586 (7): 1823-1832. 1 April 2008

Determinants of anion-proton coupling in mammalian endosomal CLC proteins.
Zdebik, A.A. and Zifarelli, G. and Bergsdorf, E.Y. and Soliani, P. and Scheel, O. and Jentsch, T.J. and Pusch, M.
Journal of Biological Chemistry 283 (7): 4219-4227. 15 February 2008

CLC chloride channels and transporters: from genes to protein structure, pathology and physiology.
Jentsch, T.J.
Critical Reviews in Biochemistry and Molecular Biology 43 (1): 3-36. January 2008

Channelopathies of transepithelial transport and vesicular function.
Huebner, C.A. and Jentsch, T.J.
Advances in Genetics 63 : 113-152. 2008

2007

Detection and differentiation of sensorineural hearing loss in mice using auditory steady-state responses and transient auditory brainstem responses.
Pauli-Magnus, D. and Hoch, G. and Strenzke, N. and Anderson, S. and Jentsch, T.J. and Moser, T.
Neuroscience 149 (3): 673-684. 9 November 2007

Leukoencephalopathy upon disruption of the chloride channel ClC-2.
Blanz, J. and Schweizer, M. and Auberson, M. and Maier, H. and Muenscher, A. and Huebner, C.A. and Jentsch, T.J.
Journal of Neuroscience 27 (24): 6581-9. 13 June 2007

Disruption of erythroid K-Cl cotransporters alters erythrocyte volume and partially rescues erythrocyte dehydration in SAD mice.
Rust, M.B. and Alper, S.L. and Rudhard, Y. and Shmukler, B.E. and Vicente, R. and Brugnara, C. and Trudel, M. and Jentsch, T.J. and Huebner, C.A.
Journal of Clinical Investigation 117 (6): 1708-1717. 1 June 2007

Chloride and the endosomal/lysosomal pathway: emerging roles of CLC chloride transporters.
Jentsch, T.J.
Journal of Physiology 578 (Pt 3): 633-640. February 2007

2006

Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.
Poet, M. and Kornak, U. and Schweizer, M. and Zdebik, A.A. and Scheel, O. and Hoelter, S. and Wurst, W. and Schmitt, A. and Fuhrmann, J.C. and Planells-Cases, R. and Mole, S.E. and Huebner, C.A. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 103 (37): 13854-13859. 12 September 2006

Kidney-specific upregulation of vitamin D3 target genes in ClC-5 KO mice.
Maritzen, T. and Rickheit, G. and Schmitt, A. and Jentsch, T.J.
Kidney International 70 (1): 79-87. 1 July 2006

Structural determinants of M-type KCNQ (Kv7) K+ channel assembly.
Schwake, M. and Athanasiadu, D. and Beimgraben, C. and Blanz, J. and Beck, C. and Jentsch, T.J. and Saftig, P. and Friedrich, T.
Journal of Neuroscience 26 (14): 3757-3766. 5 April 2006

ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function.
Lange, P.F. and Wartosch, L. and Jentsch, T.J. and Fuhrmann, J.C.
Nature 440 (7081): 220-223. 9 March 2006

Neurogenic mechanisms contribute to hypertension in mice with disruption of the K-Cl cotransporter KCC3.
Rust, M.B. and Faulhaber, J. and Budack, M.K. and Pfeffer, P. and Maritzen, T. and Didie, M. and Beck, F.X. and Boettger, T. and Schubert, R. and Ehmke, H. and Jentsch, T.J. and Huebner, C.A.
Circulation Research 98 (4): 549-556. 3 March 2006

Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness.
Kharkovets, T. and Dedek, K. and Maier, H. and Schweizer, M. and Khimich, D. and Nouvian, R. and Vardanyan, V. and Leuwer, R. and Moser, T. and Jentsch, T.J.
EMBO Journal 25 (3): 642-652. 8 February 2006

Mice with a targeted disruption of the Cl-/HCO3- exchanger AE3 display a reduced seizure threshold.
Hentschke, M. and Wiemann, M. and Hentschke, S. and Kurth, I. and Hermans-Borgmeyer, I. and Seidenbecher, T. and Jentsch, T.J. and Gal, A. and Huebner, C.A.
Molecular and Cellular Biology 26 (1): 182-191. January 2006

2005

Chloride channel diseases resulting from impaired transepithelial transport or vesicular function.
Jentsch, T.J. and Maritzen, T. and Zdebik, A.A.
Journal of Clinical Investigation 115 (8): 2039-2046. August 2005

Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins.
Scheel, O. and Zdebik, A.A. and Lourdel, S. and Jentsch, T.J.
Nature 436 (7049): 424-427. 21 July 2005

CLC chloride channels and transporters.
Jentsch, T.J. and Neagoe, I. and Scheel, O.
Current Opinion in Neurobiology 15 (3): 319-325. June 2005

Chloride transport in the kidney: lessons from human disease and knockout mice.
Jentsch, T.J.
Journal of the American Society of Nephrology 16 (6): 1549-1561. June 2005

Molecular determinants of KCNQ (Kv7) K+ channel sensitivity to the anticonvulsant retigabine.
Schenzer, A. and Friedrich, T. and Pusch, M. and Saftig, P. and Jentsch, T.J. and Groetzinger, J. and Schwake, M.
Journal of Neuroscience 25 (20): 5051-5060. 18 May 2005

Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration.
Kasper, D. and Planells-Cases, R. and Fuhrmann, J.C. and Scheel, O. and Zeitz, O. and Ruether, K. and Schmitt, A. and Poet, M. and Steinfeld, R. and Schweizer, M. and Kornak, U. and Jentsch, T.J.
EMBO Journal 24 (5): 1079-1091. 9 March 2005

Physiological functions of CLC Cl- channels gleaned from human genetic disease and mouse models.
Jentsch, T.J. and Poet, M. and Fuhrmann, J.C. and Zdebik, A.A.
Annual Review of Physiology 67 : 779-807. March 2005

Unique structure and function of chloride transporting CLC proteins.
Pusch, M. and Jentsch, T.J.
IEEE Transactions on Nanobioscience 4 (1): 49-57. March 2005

2004

Ion channels: function unravelled by dysfunction.
Jentsch, T.J. and Huebner, C.A. and Fuhrmann, J.C.
Nature Cell Biology 6 (11): 1039-1047. November 2004

Plasmodium induces swelling-activated ClC-2 anion channels in the host erythrocyte.
Huber, S.M. and Duranton, C. and Henke, G. and Van De Sand, C. and Heussler, V. and Shumilina, E. and Sandu, C.D. and Tanneur, V. and Brand, V. and Kasinathan, R.S. and Lang, K.S. and Kremsner, P.G. and Huebner, C.A. and Rust, M.B. and Dedek, K. and Jentsch, T.J. and Lang, F.
Journal of Biological Chemistry 279 (40): 41444-41452. 1 October 2004

Functional and structural conservation of CBS domains from CLC chloride channels.
Estevez, R. and Pusch, M. and Ferrer-Costa, C. and Orozco, M. and Jentsch, T.J.
Journal of Physiology 557 (Pt 2): 363-378. 1 June 2004

Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models.
Zdebik, A.A. and Cuffe, J.E. and Bertog, M. and Korbmacher, C. and Jentsch, T.J.
Journal of Biological Chemistry 279 (21): 22276-22283. 21 May 2004

Expression of the KCl cotransporter KCC2 parallels neuronal maturation and the emergence of low intracellular chloride.
Stein, V. and Hermans-Borgmeyer, I. and Jentsch, T.J. and Huebner, C.A.
Journal of Comparative Neurology 468 (1): 57-64. 1 January 2004

2003

Loss of K-Cl co-transporter KCC3 causes deafness, neurodegeneration and reduced seizure threshold.
Boettger, T. and Rust, M.B. and Maier, H. and Seidenbecher, T. and Schweizer, M. and Keating, D.J. and Faulhaber, J. and Ehmke, H. and Pfeffer, C. and Scheel, O. and Lemcke, B. and Horst, J. and Leuwer, R. and Pape, H.C. and Voelkl, H. and Huebner, C.A. and Jentsch, T.J.
EMBO Journal 22 (20): 5422-5434. 15 October 2003

Conservation of chloride channel structure revealed by an inhibitor binding site in ClC-1.
Estevez, R. and Schroeder, B.C. and Accardi, A. and Jentsch, T.J. and Pusch, M.
Neuron 38 (1): 47-59. 10 April 2003

A carboxy-terminal domain determines the subunit specificity of KCNQ K+ channel assembly.
Schwake, M. and Jentsch, T.J. and Friedrich, T.
EMBO Reports 4 (1): 76-81. January 2003

The ClC-5 chloride channel knock-out mouse - an animal model for Dent's disease.
Guenther, W. and Piwon, N. and Jentsch, T.J.
Pfluegers Archiv European Journal of Physiology 445 (4): 456-462. January 2003

2002

Ion channel diseases.
Huebner, C.A. and Jentsch, T.J.
Human Molecular Genetics 11 (20): 2435-2445. 1 October 2002

CLC chloride channels: correlating structure with function.
Estevez, R. and Jentsch, T.J.
Current Opinion in Structural Biology 12 (4): 531-539. August 2002

Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4.
Boettger, T. and Huebner, C.A. and Maier, H. and Rust, M.B. and Beck, F.X. and Jentsch, T.J.
Nature 416 (6883): 874-878. 25 April 2002

Molecular structure and physiological function of chloride channels.
Jentsch, T.J. and Stein, V. and Weinreich, F. and Zdebik, A.A.
Physiological Reviews 82 (2): 503-568. April 2002

Chloride channels are different.
Jentsch, T.J.
Nature 415 (6869): 276-277. 17 January 2002

2001

Barttin is a Cl- channel beta-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion.
Estevez, R. and Boettger, T. and Stein, V. and Birkenhaeger, R. and Otto, E. and Hildebrandt, F. and Jentsch, T.J.
Nature 414 (6863): 558-561. 29 November 2001

Planned law could put German research at risk.
Jentsch, T.
Nature 413 (6858): 772. 25 October 2001

Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel.
Dedek, K. and Kunath, B. and Kananura, C. and Reuner, U. and Jentsch, T.J. and Steinlein, O.K.
Proceedings of the National Academy of Sciences of the United States of America 98 (21): 12272-12277. 9 October 2001

Mutations in CAV3 cause mechanical hyperirritability of skeletal muscle in rippling muscle disease.
Betz, R.C. and Schoser, B.G.H. and Kasper, D. and Ricker, K. and Ramirez, A. and Stein, V. and Torbergsen, T. and Lee, Y.A. and Nothen, M.M. and Wienker, T.F. and Malin, J.P. and Propping, P. and Reis, A. and Mortier, W. and Jentsch, T.J. and Vorgerd, M. and Kubisch, C.
Nature Genetics 28 (3): 218-219. July 2001

An internalization signal in ClC-5, an endosomal Cl-channel mutated in dent's disease.
Schwake, M. and Friedrich, T. and Jentsch, T.J.
Journal of Biological Chemistry 276 (15): 12049-12054. 13 April 2001

Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption.
Boesl, M.R. and Stein, V. and Huebner, C.A. and Zdebik, A.A. and Jordt, S.E. and Mukhopadhyay, A.K. and Davidoff, M.S. and Holstein, A.F. and Jentsch, T.J.
EMBO Journal 20 (6): 1289-1299. 15 March 2001

Disruption of KCC2 reveals an essential role of K-Cl cotransport already in early synaptic inhibition.
Huebner, C.A. and Stein, V. and Hermans-Borgmeyer, I. and Meyer, T. and Ballanyi, K. and Jentsch, T.J.
Neuron 30 (2): 515-524. March 2001

Tissue distribution and subcellular localization of the ClC-5 chloride channel in rat intestinal cells.
Vandewalle, A. and Cluzeaud, F. and Peng, K.C. and Bens, M. and Luechow, A. and Guenther, W. and Jentsch, T.J.
American Journal of Physiology Cell Physiology 280 (2): C373-C381. February 2001

Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man.
Kornak, U. and Kasper, D. and Boesl, M.R. and Kaiser, E. and Schweizer, M. and Schulz, A. and Friedrich, W. and Delling, G. and Jentsch, T.J.
Cell 104 (2): 205-215. 26 January 2001

Pores formed by single subunits in mixed dimers of different CLC chloride channels.
Weinreich, F. and Jentsch, T.J.
Journal of Biological Chemistry 276 (4): 2347-2353. 26 January 2001

Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus.
Stobrawa, S.M. and Breiderhoff, T. and Takamori, S. and Engel, D. and Schweizer, M. and Zdebik, A.A. and Boesl, M.R. and Ruether, K. and Jahn, H. and Draguhn, A. and Jahn, R. and Jentsch, T.J.
Neuron 29 (1): 185-196. January 2001

2000

ClC-5 Cl- -channel disruption impairs endocytosis in a mouse model for Dent's disease.
Piwon, N. and Guenther, W. and Schwake, M. and Boesl, M.R. and Jentsch, T.J.
Nature 408 (6810): 369-373. 16 November 2000

Neuronal KCNQ potassium channels: physiology and role in disease.
Jentsch, T.J.
Nature Reviews Neuroscience 1 (1): 21-30. October 2000

Mutations in the a3 subunit of the vacuolar H(+)-ATPase cause infantile malignant osteopetrosis.
Kornak, U. and Schulz, A. and Friedrich, W. and Uhlhaas, S. and Kremens, B. and Voit, T. and Hasan, C. and Bode, U. and Jentsch, T.J. and Kubisch, C.
Human Molecular Genetics 9 (13): 2059-2063. 12 August 2000

Functional and structural analysis of ClC-K chloride channels involved in renal disease.
Waldegger, S. and Jentsch, T.J.
Journal of Biological Chemistry 275 (32): 24527-24533. 11 August 2000

KCNQ5, a novel potassium channel broadly expressed in brain, mediates M-type currents.
Schroeder, B.C. and Hechenberger, M. and Weinreich, F. and Kubisch, C. and Jentsch, T.J.
Journal of Biological Chemistry 275 (31): 24089-24095. 4 August 2000

Characterization of renal chloride channel (CLCN5) mutations in Dent's disease.
Yamamoto, K. and Cox, J.P. and Friedrich, T. and Christie, P.T. and Bald, M. and Houtman, P.N. and Lapsley, M.J. and Patzer, L. and Tsimaratos, M. and Van'T Hoff, W.G. and Yamaoka, K. and Jentsch, T.J. and Thakker, R.V.
Journal of the American Society of Nephrology 11 (8): 1460-1468. August 2000

Pathophysiology of KCNQ channels: neonatal epilepsy and progressive deafness.
Jentsch, T.J. and Schroeder, B.C. and Kubisch, C. and Friedrich, T. and Stein, V.
Epilepsia 41 (8): 1068-1069. August 2000

A function for dysfunction - Ion channels and disease.
Jentsch, T.J.
Nature 406 (6793): 236-237. 20 July 2000

From tonus to tonicity: physiology of CLC chloride channels.
Waldegger, S. and Jentsch, T.J.
Journal of the American Society of Nephrology 11 (7): 1331-1339. July 2000

Neurological diseases caused by ion-channel mutations.
Weinreich, F. and Jentsch, T.J.
Current Opinion in Neurobiology 10 (3): 409-415. June 2000

Surface expression and single channel properties of KCNQ2/KCNQ3, M-type K+ channels involved in epilepsy.
Schwake, M. and Pusch, M. and Kharkovets, T. and Jentsch, T.J.
Journal of Biological Chemistry 275 (18): 13343-13348. 5 May 2000

KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.
Kharkovets, T. and Hardelin, J.P. and Safieddine, S. and Schweizer, M. and El-Amraoui, A. and Petit, C. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 97 (8): 4333-4338. 11 April 2000

Inhibition of KCNQ1-4 potassium channels expressed in mammalian cells via M1 muscarinic acetylcholine receptors.
Selyanko, A.A. and Hadley, J.K. and Wood, I.C. and Abogadie, F.C. and Jentsch, T.J. and Brown, D.A.
Journal of Physiology 522 (Pt 3): 349-355. 1 February 2000

A constitutively open potassium channel formed by KCNQ1 and KCNE3.
Schroeder, B.C. and Waldegger, S. and Fehr, S. and Bleich, M. and Warth, R. and Greger, R. and Jentsch, T.J.
Nature 403 (6766): 196-199. 13 January 2000

1999

CLC chloride channels in Caenorhabditis elegans.
Schriever, A.M. and Friedrich, T. and Pusch, M. and Jentsch, T.J.
Journal of Biological Chemistry 274 (48): 34238-34244. 26 November 1999

The CLC chloride channel family.
Jentsch, T.J. and Friedrich, T. and Schriever, A. and Yamada, H.
Pfluegers Archiv European Journal of Physiology 437 (6): 783-795. May 1999

Chloride dependence of hyperpolarization-activated chloride channel gates.
Pusch, M. and Jordt, S.E. and Stein, V. and Jentsch, T.J.
Journal of Physiology 515 (Pt 2): 341-353. 1 March 1999

KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness.
Kubisch, C. and Schroeder, B.C. and Friedrich, T. and Luetjohann, B. and El-Amraoui, A. and Marlin, S. and Petit, C. and Jentsch, T.J.
Cell 96 (3): 437-446. 5 February 1999

Mutational analysis demonstrates that ClC-4 and ClC-5 directly mediate plasma membrane currents.
Friedrich, T. and Breiderhoff, T. and Jentsch, T.J.
Journal of Biological Chemistry 274 (2): 896-902. 8 January 1999

1998

Moderate loss of function of cyclic-AMP-modulated KCNQ2/KCNQ3 K+ channels causes epilepsy.
Schroeder, B.C. and Kubisch, C. and Stein, V. and Jentsch, T.J.
Nature 396 (6712): 687-690. 17 December 1998

Functional characterization of renal chloride channel, CLCN5, mutations associated with Dent'sJapan disease.
Igarashi, T. and Guenther, W. and Sekine, T. and Inatomi, J. and Shiraga, H. and Takahashi, S. and Suzuki, J. and Tsuru, N. and Yanagihara, T. and Shimazu, M. and Jentsch, T.J. and Thakker, R.V.
Kidney International 54 (6): 1850-1856. December 1998

KCNQ2, the first gene found to be mutated in human generalized idiopathic epilepsy.
Steinlein, O.K. and Jentsch, T.J.
Pathologie Biologie 46 (9): 683-684. November 1998

ClC-1 chloride channel mutations in myotonia congenita: variable penetrance of mutations shifting the voltage dependence.
Kubisch, C. and Schmidt-Rose, T. and Fontaine, B. and Bretag, A.H. and Jentsch, T.J.
Human Molecular Genetics 7 (11): 1753-1760. October 1998

Molecular physiology of renal chloride channels.
Steinmeyer, K. and Jentsch, T.J.
Current Opinion in Nephrology and Hypertension 7 (5): 497-502. September 1998

ClC-5, the chloride channel mutated in Dent's disease, colocalizes with the proton pump in endocytotically active kidney cells.
Guenther, W. and Luechow, A. and Cluzeaud, F. and Vandewalle, A. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 95 (14): 8075-8080. 7 July 1998

Golgi localization and functionally important domains in the NH2 and COOH terminus of the yeast CLC putative chloride channel Gef1p.
Schwappach, B. and Stobrawa, S. and Hechenberger, M. and Steinmeyer, K. and Jentsch, T.J.
Journal of Biological Chemistry 273 (24): 15110-15108. 12 June 1998

Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions.
Rychkov, G.Y. and Pusch, M. and Roberts, M.L. and Jentsch, T.J. and Bretag, A.H.
Journal of General Physiology 111 (5): 653-665. May 1998

Determinants of slow gating in ClC-0, the voltage-gated chloride channel of Torpedo marmorata.
Fong, P. and Rehfeldt, A. and Jentsch, T.J.
American Journal of Physiology 274 (4 Pt 1): C966-C973. April 1998

Novel muscle chloride channel (CLCN1) mutations in myotonia congenita with various modes of inheritance including incomplete dominance and penetrance.
Plassart-Schiess, E. and Gervais, A. and Eymard, B. and Lagueny, A. and Pouget, J. and Warter, J.M. and Fardeau, M. and Jentsch, T.J. and Fontaine, B.
Neurology 50 (4): 1176-1179. April 1998

Characterization of the hyperpolarization-activated chloride current in dissociated rat sympathetic neurons.
Clark, S. and Jordt, S.E. and Jentsch, T.J. and Mathie, A.
Journal of Physiology 506 (Pt 3): 665-678. 1 February 1998

A potassium channel mutation in neonatal human epilepsy.
Biervert, C. and Schroeder, B.C. and Kubisch, C. and Berkovic, S.F. and Propping, P. and Jentsch, T.J. and Steinlein, O.K.
Science 279 (5349): 403-406. 16 January 1998

Molecular diagnosis of McArdle disease: revised genomic structure of the myophosphorylase gene and identification of a novel mutation.
Kubisch, C. and Wicklein, E.M. and Jentsch, T.J.
Human Mutation 12 (1): 27-32. 1998

1997

Pathophysiological mechanisms of dominant and recessive KVLQT1 K+ channel mutations found in inherited cardiac arrhythmias.
Wollnik, B. and Schroeder, B.C. and Kubisch, C. and Esperer, H.D. and Wieacker, P. and Jentsch, T.J.
Human Molecular Genetics 6 (11): 1943-1949. October 1997

Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1.
Schmidt-Rose, T. and Jentsch, T.J.
Journal of Biological Chemistry 272 (33): 20515-20521. 15 August 1997

Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders.
Lloyd, S.E. and Gunther, W. and Pearce, S.H. and Thomson, A. and Bianchi, M.L. and Bosio, M. and Craig, I.W. and Fisher, S.E. and Scheinman, S.J. and Wrong, O. and Jentsch, T.J. and Thakker, R.V.
Human Molecular Genetics 6 (8): 1233-1239. August 1997

Independent gating of single pores in CLC-0 chloride channels.
Ludewig, U. and Pusch, M. and Jentsch, T.J.
Biophysical Journal 73 (2): 789-797. August 1997

Inward rectification in ClC-0 chloride channels caused by mutations in several protein regions.
Ludewig, U. and Jentsch, T.J. and Pusch, M.
Journal of General Physiology 110 (2): 165-171. August 1997

Transmembrane topology of a CLC chloride channel.
Schmidt-Rose, T. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 94 (14): 7633-7638. 8 July 1997

Localization and induction by dehydration of ClC-K chloride channels in the rat kidney.
Vandewalle, A. and Cluzeaud, F. and Bens, M. and Kieferle, S. and Steinmeyer, K. and Jentsch, T.J.
American Journal of Physiology 272 (5 Pt 2): F678-F688. May 1997

Molecular dissection of gating in the ClC-2 chloride channel.
Jordt, S.E. and Jentsch, T.J.
EMBO Journal 16 (7): 1582-1592. 1 April 1997

Idiopathic low molecular weight proteinuria associated with hypercalciuric nephrocalcinosis in Japanese children is due to mutations of the renal chloride channel (CLCN5).
Lloyd, S.E. and Pearce, S.H. and Guenther, W. and Kawaguchi, H. and Igarashi, T. and Jentsch, T.J. and Thakker, R.V.
Journal of Clinical Investigation 99 (5): 967-974. 1 March 1997

Analysis of a protein region involved in permeation and gating of the voltage-gated Torpedo chloride channel ClC-0.
Ludewig, U. and Jentsch, T.J. and Pusch, M.
Journal of Physiology 498 (Pt 3): 691-702. 1 February 1997

Chloride channels: an emerging molecular picture.
Jentsch, T.J. and Guenther, W.
BioEssays 19 (2): 117-126. February 1997

Temperature dependence of fast and slow gating relaxations of ClC-0 chloride channels.
Pusch, M. and Ludewig, U. and Jentsch, T.J.
Journal of General Physiology 109 (1): 105-116. January 1997

1996

A family of putative chloride channels from Arabidopsis and functional complementation of a yeast strain with a CLC gene disruption.
Hechenberger, M. and Schwappach, B. and Fischer, W.N. and Frommer, W.B. and Jentsch, T.J. and Steinmeyer, K.
Journal of Biological Chemistry 271 (52): 33632-33638. 27 December 1996

Concentration and pH dependence of skeletal muscle chloride channel ClC-1.
Rychkov, G.Y. and Pusch, M. and Astill, D.S. and Roberts, M.L. and Jentsch, T.J. and Bretag, A.H.
Journal of Physiology 497 (Pt 2): 423-435. 1 December 1996

Heteromultimeric CLC chloride channels with novel properties.
Lorenz, C. and Pusch, M. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 93 (23): 13362-13366. 12 November 1996

Two physically distinct pores in the dimeric ClC-0 chloride channel.
Ludewig, U. and Pusch, M. and Jentsch, T.J.
Nature 383 (6598): 340-343. 26 September 1996

Alteration of GABAA receptor function following gene transfer of the CLC-2 chloride channel.
Staley, K. and Smith, R. and Schaack, J. and Wilcox, C. and Jentsch, T.J.
Neuron 17 (3): 543-551. September 1996

Chloride Channel Myotonias.
Steinmeyer, K. and Jentsch, T.J.
Neuroscientist 2 (4): 225-232. July 1996

Chloride channels: a molecular perspective.
Jentsch, T.J.
Current Opinion in Neurobiology 6 (3): 303-310. June 1996

A common molecular basis for three inherited kidney stone diseases.
Lloyd, S.E. and Pearce, S.H. and Fisher, S.E. and Steinmeyer, K. and Schwappach, B. and Scheinman, S.J. and Harding, B. and Bolino, A. and Devoto, M. and Goodyer, P. and Rigden, S.P. and Wrong, O. and Jentsch, T.J. and Craig, I.W. and Thakker, R.V.
Nature 379 (6564): 445-449. 1 February 1996

1995

Cloning and functional expression of rat CLC-5, a chloride channel related to kidney disease.
Steinmeyer, K. and Schwappach, B. and Bens, M. and Vandewalle, A. and Jentsch, T.J.
Journal of Biological Chemistry 270 (52): 31172-31177. 29 December 1995

ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family.
Brandt, S. and Jentsch, T.J.
FEBS Letters 377 (1): 15-20. 11 December 1995

Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the CIC-1 chloride channel.
Pusch, M. and Steinmeyer, K. and Koch, M.C. and Jentsch, T.J.
Neuron 15 (6): 1455-1463. December 1995

Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCN1) leading to myotonia.
Meyer-Kleine, C. and Steinmeyer, K. and Ricker, K. and Jentsch, T.J. and Koch, M.C.
American Journal of Human Genetics 57 (6): 1325-1334. December 1995

Chloride channel 2 gene (Clc2) maps to chromosome 16 of the mouse, extending a region of conserved synteny with human chromosome 3q.
Lengeling, A. and Gronemeier, M. and Ronsiek, M. and Thiemann, A. and Jentsch, T.J. and Jockusch, H.
Genetical Research 66 (2): 175-178. October 1995

Molecular basis of epithelial Cl channels.
Fong, P. and Jentsch, T.J.
Journal of Membrane Biology 144 (3): 189-197. April 1995

Gating of the voltage-dependent chloride channel CIC-0 by the permeant anion.
Pusch, M. and Ludewig, U. and Rehfeldt, A. and Jentsch, T.J.
Nature 373 (6514): 527-531. 9 February 1995

Properties of voltage-gated chloride channels of the ClC gene family.
Jentsch, T.J. and Guenther, W. and Pusch, M. and Schwappach, B.
Journal of Physiology 482 : 19S-25S. January 1995

Myotonias due to CLC-1 chloride channel mutations.
Jentsch, T.J. and Lorenz, C. and Pusch, M. and Steinmeyer, K.
Society of General Physiologists Series 50 : 149-159. 1995

1994

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. and Steinmeyer, K. and Jentsch, T.J. and Jockusch, H.
Journal of Biological Chemistry 269 (44): 27635-27639. 4 November 1994

Molecular physiology of voltage-gated chloride channels.
Pusch, M. and Jentsch, T.J.
Physiological Reviews 74 (4): 813-827. October 1994

Molecular physiology of anion channels.
Jentsch, T.J.
Current Opinion in Cell Biology 6 (4): 600-606. August 1994

Two highly homologous members of the ClC chloride channel family in both rat and human kidney.
Kieferle, S. and Fong, P. and Bens, M. and Vandewalle, A. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 91 (15): 6943-6947. 19 July 1994

Genomic organization of the human muscle chloride channel CIC-1 and analysis of novel mutations leading to Becker-type myotonia.
Lorenz, C. and Meyer-Kleine, C. and Steinmeyer, K. and Koch, M.C. and Jentsch, T.J.
Human Molecular Genetics 3 (6): 941-946. June 1994

Nonsense and missense mutations in the muscular chloride channel gene Clc-1 of myotonic mice.
Gronemeier, M. and Condie, A. and Prosser, J. and Steinmeyer, K. and Jentsch, T.J. and Jockusch, H.
Journal of Biological Chemistry 269 (8): 5963-5967. 25 February 1994

Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen).
Steinmeyer, K. and Lorenz, C. and Pusch, M. and Koch, M.C. and Jentsch, T.J.
EMBO Journal 13 (4): 737-743. 15 February 1994

Trinity of cation channels.
Jentsch, T.J.
Nature 367 (6462): 412-413. 3 February 1994

Low single channel conductance of the major skeletal muscle chloride channel, ClC-1.
Pusch, M. and Steinmeyer, K. and Jentsch, T.J.
Biophysical Journal 66 (1): 149-152. January 1994

Molecular biology of voltage-gated chloride channels.
Jentsch, T.J.
Current Topics in Membranes 42 : 35-57. 1994

Structure and function of ClC chloride channels.
Jentsch, T.J.
Japanese Journal of Physiology 44 (Suppl 2): S1-S2. 1994

1993

The ClC family of voltage-gated chloride channels: structure and function.
Jentsch, T.J. and Pusch, M. and Rehfeldt, A. and Steinmeyer, K.
Annals of the New York Academy of Sciences 707 : 285-293. 20 December 1993

Evidence for genetic homogeneity in autosomal recessive generalised myotonia (Becker).
Koch, M.C. and Ricker, K. and Otto, M. and Wolf, F. and Zoll, B. and Lorenz, C. and Steinmeyer, K. and Jentsch, T.J.
Journal of Medical Genetics 30 (11): 914-917. November 1993

Chloride channels.
Jentsch, T.J.
Current Opinion in Neurobiology 3 (3): 316-321. June 1993

1992

Regions involved in the opening of CIC-2 chloride channel by voltage and cell volume.
Gruender, S. and Thiemann, A. and Pusch, M. and Jentsch, T.J.
Nature 360 (6406): 759-762. 24 December 1992

The skeletal muscle chloride channel in dominant and recessive human myotonia.
Koch, M.C. and Steinmeyer, K. and Lorenz, C. and Ricker, K. and Wolf, F. and Otto, M. and Zoll, B. and Lehmann-Horn, F. and Grzeschik, K.H. and Jentsch, T.J.
Science 257 (5071): 797-800. 7 August 1992

A chloride channel widely expressed in epithelial and non-epithelial cells.
Thiemann, A. and Gruender, S. and Pusch, M. and Jentsch, T.J.
Nature 356 (6364): 57-60. 5 March 1992

1991

Completely functional double-barreled chloride channel expressed from a single Torpedo cDNA.
Bauer, C.K. and Steinmeyer, K. and Schwarz, J.R. and Jentsch, T.J.
Proceedings of the National Academy of Sciences of the United States of America 88 (24): 11052-11056. 15 December 1991

Inactivation of muscle chloride channel by transposon insertion in myotonic mice.
Steinmeyer, K. and Klocke, R. and Ortland, C. and Gronemeier, M. and Jockusch, H. and Gruender, S. and Jentsch, T.J.
Nature 354 (6351): 304-308. 28 November 1991

Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel.
Steinmeyer, K. and Ortland, C. and Jentsch, T.J.
Nature 354 (6351): 301-304. 28 November 1991

1990

Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes.
Jentsch, T.J. and Steinmeyer, K. and Schwarz, G.
Nature 348 (6301): 510-514. 6 December 1990

Cell culture of bovine corneal endothelial cells and its application to transport studies.
Wiederholt, M. and Jentsch, T.J.
Methods in Enzymology 192 : 571-582. 1990

1989

Primary structure of a novel 4-acetamido-4'-isothiocyanostilbene-2,2'-disulphonic acid (SITS)-binding membrane protein highly expressed in Torpedo californica electroplax.
Jentsch, T.J. and Garcia, A.M. and Lodish, H.F.
Biochemical Journal 261 (1): 155-166. 1 July 1989

1988

Regulation of cytoplasmic pH of cultured bovine corneal endothelial cells in the absence and presence of bicarbonate.
Jentsch, T.J. and Korbmacher, C. and Janicke, I. and Fischer, D.G. and Stahl, F. and Helbig, H. and Hollwede, H. and Cragoe, E.J. and Keller, S.K. and Wiederholt, M.
Journal of Membrane Biology 103 (1): 29-40. July 1988

Regulation of intracellular pH in cultured bovine retinal pigment epithelial cells.
Keller, S.K. and Jentsch, T.J. and Janicke, I. and Wiederholt, M.
Pfluegers Archiv European Journal of Physiology 411 (1): 47-52. January 1988

1986

Electrical properties of sodium bicarbonate symport in kidney epithelial cells (BSC-1).
Jentsch, T.J. and Matthes, H. and Keller, S.K. and Wiederholt, M.
American Journal of Physiology 251 (6 Pt 2): F954-F968. December 1986

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, T.J. and Janicke, I. and Sorgenfrei, D. and Keller, S.K. and Wiederholt, M.
Journal of Biological Chemistry 261 (26): 12120-12127. 15 September 1986

Kinetic properties of the sodium bicarbonate (carbonate) symport in monkey kidney epithelial cells (BSC-1). Interactions between Na+, HCO-3, and pH.
Jentsch, T.J. and Schwartz, P. and Schill, B.S. and Langner, B. and Lepple, A.P. and Keller, S.K. and Wiederholt, M.
Journal of Biological Chemistry 261 (23): 10673-10679. 15 August 1986

Interactions of pH and K+ conductance in cultured bovine retinal pigment epithelial cells.
Keller, S.K. and Jentsch, T.J. and Koch, M. and Wiederholt, M.
American Journal of Physiology 250 (1 Pt 1): C124-C137. January 1986

1985

Kidney epithelial cells of monkey origin (BSC-1) express a sodium bicarbonate cotransport. Characterization by 22Na+ flux measurements.
Jentsch, T.J. and Schill, B.S. and Schwartz, P. and Matthes, H. and Keller, S.K. and Wiederholt, M.
Journal of Biological Chemistry 260 (29): 15554-15560. 15 December 1985

Response of the intracellular potentials of cultured bovine lens cells to ions and inhibitors.
Jentsch, T.J. and von der Haar, B. and Keller, S.K. and Wiederholt, M.
Experimental Eye Research 41 (2): 131-144. August 1985

Observation of doubly charged triatomic cluster ions in field evaporation.
Drachsel, W. and Jentsch, T.J. and Gingerich, K.A. and Block, J.H.
Surface Science 156 (Pt 1): 173-182. 3 June 1985

Ion transport mechanisms in cultured bovine corneal endothelial cells.
Jentsch, T.J. and Keller, S.K. and Wiederholt, M.
Current Eye Research 4 (4): 361-369. April 1985

Electrogenic sodium-bicarbonate symport in cultured corneal endothelial cells.
Wiederholt, M. and Jentsch, T.J. and Keller, S.K.
Pfluegers Archiv European Journal of Physiology 405 (Suppl 1): S167-S171. March 1985

Anion dependence of electrical effects of bicarbonate and sodium on cultured bovine corneal endothelial cells.
Jentsch, T.J. and Matthes, H. and Keller, S.K. and Wiederholt, M.
Pfluegers Archiv European Journal of Physiology 403 (2): 175-185. February 1985

A bicarbonate-dependent process inhibitable by disulfonic stilbenes and a Na+/H+ exchange mediate 22Na+ uptake into cultured bovine corneal endothelium.
Jentsch, T.J. and Stahlknecht, T.R. and Hollwede, H. and Fischer, D.G. and Keller, S.K. and Wiederholt, M.
Journal of Biological Chemistry 260 (2): 795-801. 25 January 1985

1984

Evidence for coupled transport of bicarbonate and sodium in cultured bovine corneal endothelial cells.
Jentsch, T.J. and Keller, S.K. and Koch, M. and Wiederholt, M.
Journal of Membrane Biology 81 (3): 189-204. October 1984

The calcium antagonist nisoldipine stimulates the electrolyte transport of the isolated frog skin.
Wiederholt, M. and Keller, S. and Krolik, A. and Jentsch, T.J.
Biochemical Pharmacology 33 (18): 2926-2928. 15 September 1984

Effect of bicarbonate, pH, methazolamide and stilbenes on the intracellular potentials of cultured bovine corneal endothelial cells.
Jentsch, T.J. and Koch, M. and Bleckmann, H. and Wiederholt, M.
Journal of Membrane Biology 78 (2): 103-117. June 1984

1983

New techniques in surface specific mass analysis by photon induced field desorption.
Drachsel, W. and Jentsch, T.J. and Block, J.H.
International Journal of Mass Spectrometry and Ion Physics 46 : 293-296. January 1983

1982

Stability of doubly charged homonuclear trimeric metal clusters.
Jentsch, T.J. and Drachsel, W. and Block, J.H.
Chemical Physics Letters 93 (2): 144-147. 26 November 1982

1981

Post-field ionization of singly charged rhodium: An experimental and theoretical study.
Ernst, N. and Jentsch, T.J.
Physical Review B 24 (11): 6234-6241. July 1981

Copper cluster ions in photon-induced field ionization mass spectra.
Jentsch, T.J. and Drachsel, W. and Block, J.H.
International Journal of Mass Spectrometry and Ion Physics 38 (2-3): 215-222. May 1981

Signal shapes observed in photon-induced field ionization mass spectra.
Block, J.H. and Jentsch, T.J. and Drachsel, W.
International Journal of Mass Spectrometry and Ion Physics 38 (2-3): 195-213. May 1981

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