Thomas Jentsch

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

• Thomas J. Jentsch; Michael Pusch

Physiological Reviews 2018

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

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

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

Nature Communications 2018

K <inf>2P</inf> TASK-2 and KCNQ1–KCNE3 K ⁺ 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

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

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

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

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

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

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

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

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

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

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

• Jentsch TJ

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

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

• Schütze S; Orozco IJ; Jentsch TJ

VRAC: molecular identification as LRRC8 heteromers with differential functions.

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

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

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

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

Optogenetic acidification of synaptic vesicles and lysosomes.

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

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

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

Departure gate of acidic Ca²⁺ confirmed.

• Jentsch TJ; Hoegg-Beiler MB; Vogt J

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

• Jentsch TJ

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

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

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

• Jentsch TJ

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

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

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

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

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

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

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

From mice to man: chloride transport in leukoencephalopathy.

• Jentsch TJ

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

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

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

• Veeramah KR; Johnstone L; Karafet TM; Wolf D; Sprissler R; Salogiannis J; Barth-Maron A; Greenberg ME; Stuhlmann T; Weinert S; Jentsch TJ; Pazzi M; Restifo LL; Talwar D; Erickson RP; Hammer MF

Vestibular role of KCNQ4 and KCNQ5 K+ channels revealed by mouse models.

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

Chloride in vesicular trafficking and function.

• Stauber T; Jentsch TJ

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

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

Cell biology and physiology of CLC chloride channels and transporters.

• Stauber T; Weinert S; Jentsch TJ

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

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

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

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

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

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

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

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

Generation and analyses of R8L barttin knockin mouse.

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

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

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

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

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

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

Sorting motifs of the endosomal/lysosomal CLC chloride transporters.

• Stauber T; Jentsch TJ

A cation counterflux supports lysosomal acidification.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

• Zdebik AA; Wangemann P; Jentsch TJ

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

• Bergsdorf EY; Zdebik AA; Jentsch TJ

Chloride channelopathies.

• Planells-Cases R; Jentsch TJ

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

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

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

• Ueno, R.; Jentsch, T.J.

Critical Reviews in Biochemistry and Molecular Biology 2009

To the editor

• Jentsch, T.J.

Critical Reviews in Biochemistry and Molecular Biology 2009

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

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

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

• Plans V; Rickheit G; Jentsch TJ

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

• Jentsch TJ

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

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

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

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

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

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

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

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

Channelopathies of transepithelial transport and vesicular function.

• Hübner CA; Jentsch TJ

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

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

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

• Jentsch TJ

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

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

Leukoencephalopathy upon disruption of the chloride channel ClC-2.

• Blanz J; Schweizer M; Auberson M; Maier H; Muenscher A; Hübner CA; Jentsch TJ

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

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

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

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

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

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

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

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

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

Physiological Functions of the CLC Chloride Transport Proteins

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

Advances in Molecular and Cell Biology 2006

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

• Jentsch TJ; Maritzen T; Zdebik AA

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

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

CLC chloride channels and transporters.

• Jentsch TJ; Neagoe I; Scheel O

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

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

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

• Jentsch TJ

Unique structure and function of chloride transporting CLC proteins.

• Pusch M; Jentsch TJ

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

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

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

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

Ion channels: function unravelled by dysfunction.

• Jentsch TJ; Hübner CA; Fuhrmann JC

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

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

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

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

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

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

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

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

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

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

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

• Schwake M; Jentsch TJ; Friedrich T

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

• Günther W; Piwon N; Jentsch TJ

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

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

Physiological Reviews 2003

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

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

Pflugers Archiv European Journal of Physiology 2003

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

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

Voltage-Gated Ion Channels

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

Wiley Encyclopedia of Molecular Medicine 2002

Chloride channels are different.

• Jentsch TJ

Ion channel diseases.

• Hübner CA; Jentsch TJ

CLC chloride channels: correlating structure with function.

• Estévez R; Jentsch TJ

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

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

Molecular structure and physiological function of chloride channels.

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

Ion channel diseases

• Hübner, C.A.; Jentsch, T.J.

Human Molecular Genetics 2002

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

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

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

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

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

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

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

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

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

Planned law could put German research at risk [4]

• Jentsch, T.

Nature 2001

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

• Schwake M; Friedrich T; Jentsch TJ

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

• Weinreich F; Jentsch TJ

A constitutively open potassium channel formed by KCNQ1 and KCNE3.

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

Pathophysiology of KCNQ channels: neonatal epilepsy and progressive deafness.

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

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

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

• Waldegger S; Jentsch TJ

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

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

Neuronal KCNQ potassium channels: physiology and role in disease.

• Jentsch TJ

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

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

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

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

From tonus to tonicity: physiology of CLC chloride channels.

• Waldegger S; Jentsch TJ

Neurological diseases caused by ion-channel mutations.

• Weinreich F; Jentsch TJ

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

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

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, T.J.; Kubisch, C.

Human Molecular Genetics 2000

From tonus to tonicity: Physiology of CLC chloride channels

• Waldegger, S.; Jentsch, T.J.

Journal of the American Society of Nephrology 2000

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

CLC chloride channels in Caenorhabditis elegans.

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

The CLC chloride channel family.

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

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

• Friedrich T; Breiderhoff T; Jentsch TJ

Chloride dependence of hyperpolarization-activated chloride channel gates.

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

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

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

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

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

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

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

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

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

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

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

• Fong P; Rehfeldt A; Jentsch TJ

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

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

• Kubisch C; Wicklein EM; Jentsch TJ

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

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

Molecular physiology of renal chloride channels.

• Steinmeyer K; Jentsch TJ

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

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

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

A potassium channel mutation in neonatal human epilepsy.

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

CLC chloride channels in human disease

• Jentsch, T.J.

FASEB Journal 1997

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

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

• Jordt SE; Jentsch TJ

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

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

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

• Schmidt-Rose T; Jentsch TJ

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

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

• Ludewig U; Pusch M; Jentsch TJ

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

• Ludewig U; Jentsch TJ; Pusch M

Transmembrane topology of a CLC chloride channel.

• Schmidt-Rose T; Jentsch TJ

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

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

Chloride channels: an emerging molecular picture.

• Jentsch TJ; Günther W

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

• Pusch M; Ludewig U; Jentsch TJ

Chloride channel myotonias

• Steinmeyer, K.; Jentsch, T.J.

Neuroscientist 1996

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

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

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

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

Heteromultimeric CLC chloride channels with novel properties.

• Lorenz C; Pusch M; Jentsch TJ

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

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

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

• Ludewig U; Pusch M; Jentsch TJ

Chloride channels: a molecular perspective.

• Jentsch TJ

A common molecular basis for three inherited kidney stone diseases.

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

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

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

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

Molecular basis of epithelial Cl channels.

• Fong P; Jentsch TJ

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

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

Myotonias due to CLC-1 chloride channel mutations.

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

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

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

Myotonias due to CLC-1 chloride channel mutations.

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

Society of General Physiologists series 1995

Myotonias due to CLC-1 chloride channel mutations

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

Journal of General Physiology 1995

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

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

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

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

ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family.

• Brandt S; Jentsch TJ

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

Molecular physiology of voltage-gated chloride channels.

• Pusch M; Jentsch TJ

Molecular physiology of anion channels.

• Jentsch TJ

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

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

• Pusch M; Steinmeyer K; Jentsch TJ

Chapter 2 Molecular Biology of Voltage-Gated Chloride Channels

• Thomas J. Jentsch

Chloride Channels 1994

Structure and function of ClC chloride channels.

• Jentsch, T.J.

The Japanese journal of physiology 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.; Steinmeyer, K.; Jentsch, T.J.; Jockusch, H.

Journal of Biological Chemistry 1994

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

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

Molecular physiology of voltage-gated chloride channels

• Pusch, M.; Jentsch, T.J.

Physiological Reviews 1994

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

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

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

Trinity of cation channels.

• Jentsch TJ

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

• Jentsch TJ; Pusch M; Rehfeldt A; Steinmeyer K

Evidence for genetic homogeneity in autosomal recessive generalised myotonia (Becker).

• Koch MC; Ricker K; Otto M; Wolf F; Zoll B; Lorenz C; Steinmeyer K; Jentsch TJ

Evidence for genetic homogeneity in autosomal recessive generalised myotonia (Becker)

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

Journal of Medical Genetics 1993

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

• Jentsch, T.J.

Biomedicine and Pharmacotherapy 1993

Chloride channels.

• Jentsch TJ

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

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

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

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

Interactions of pH and K+ conductance in cultured bovine retinal pigment epithelial cells.

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

The regulation of intracellular pH in monkey kidney epithelial cells (BSC-1). Roles of Na⁺/H⁺ antiport, Na⁺-HCO<inf>3</inf>^--(NaCO<inf>3</inf>^-) symport, and Cl^-/HCO<inf>3</inf>^- exchange

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

Journal of Biological Chemistry 1986

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

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

Interactions of pH and K⁺ 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

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

Observation of doubly charged triatomic cluster ions in field evaporation

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

Surface Science 1985

Ion transport mechanisms in cultured bovine corneal endothelial cells

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

Current Eye Research 1985

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

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

Ion transport mechanisms in cultured bovine corneal endothelial cells.

• Jentsch TJ; Keller SK; Wiederholt M

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

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

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

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

Journal of Biological Chemistry 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.; Stahlknecht, T.R.; Hollwede, H.; Fischer, D.G.; Keller, S.K.; Wiederholt, M.

Journal of Biological Chemistry 1985

Electrogenic sodium-bicarbonate symport in cultured corneal endothelial cells.

• Wiederholt M; Jentsch TJ; Keller SK

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