Selected Original Publications
Reviews and Correspondences click here
Jeworutzky E., López-Hernández, T., Capdevila-Nortes X., Sirisi S., Bengtsson L., Montolio M., Zifarelli G., Müller C.S., Schulte U., Nunes V., Martínez A., Jentsch T.J., Gasull X., Pusch M., Estévez R. (2012).
GlialCAM, a protein defective in a leukodystrophy, serves as a ClC-2 Cl- channel auxilliary subunit.
Neuron 73, 951-961.
Pubmed pdf Suppl. Info Perspective
Seja P., Schonewille M., Spitzmaul G., Badura A., Klein I., Rudhard Y., Wisden W., Hübner C.A., de Zeuw C.I., Jentsch T.J. (2012).
Raising cytosolic Cl- in cerebellar granule cells affects their excitability and vestibulo-ocular learning.
EMBO J. 31,1217-1230.
Pubmed pdf Suppl. Info.
Heidenreich M., Lechner S.G., Vardanyan V., Wetzel C., Cremers C.W., De Lenheer E.M., Aránguez G., Moreno-Pelayo M.A., Jentsch T.J., Lewin G.R. (2012).
KCNQ4 K+channels tune mechanoreceptors for normal touch sensation in mouse and man.
Nature Neurosci. 15, 138-145.
Pubmed pdf Suppl. Info Suppl. Video News & Views News comment
Billig G.M., Pál B., Fidzinski P., Jentsch T.J. (2011).
Ca2+-activated Cl- channels are dispensable for olfaction.
Nature Neurosci 14, 763-749.
Pubmed pdf Suppl. Info
Leisle L., Ludwig C.F., Wagner, F.A., Jentsch, T.J., Stauber T. (2011).
ClC-7 is a slowly voltage-gated 2Cl-/H+ exchanger and requires Ostm1 for transport activity.
EMBO J. 30, 2140-2152.
Pubmed pdf Suppl. Info Suppl Video
Stauber T., Jentsch T.J. (2010).
Sorting motifs in the endosomal/lysosomal CLC chloride transporters.
J. Biol. Chem. 285, 34537-34548.
Pubmed pdf Supplementary Info
Steinberg B.E., Huynh K.K., Brodovitch A., Jabs S., Stauber T., Jentsch T.J., Grinstein S. (2010).
A cation counterflux supports lysosomal acidification.
J. Cell Biol. 189, 1171-1186.
Medline pdf Supplementary Info Highlight
Weinert S., Jabs S., Supanchart C., Schweizer M., Gimber N., Richter M., Rademann J., Stauber T., Kornak U., Jentsch T.J. (2010).
Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation.
Science 328, 1401-1403.
Medline pdf Supplementary Info Perspective
Novarino G., Weinert S., Rickheit G., Jentsch T.J. (2010).
Endosomal chloride-proton exchange rather than chloride conductance is crucial for renal endocytosis.
Science 328, 1398-1401.
Medline pdf Supplementary Info Perspective in Science Comment in NDT
Neagoe I., Stauber T., Fidzinski P., Bergsdorf E.-Y., Jentsch T.J. (2010).
The late endosomal ClC-6 mediates proton/chloride countertransport in heterologous plasma membrane expression.
J. Biol. Chem. 285, 21689-21697.
Medline pdf Supplementary Info
Tzingounis A.V., Heidenreich M., Kharkovets T., Spitzmaul G., Jensen H.S., Nicoll R.A., Jentsch T.J. (2010).
The KCNQ5 potassium channel mediates a component of the afterhyperpolarization current in mouse hippocampus.
Proc. Natl. Acad. Sci USA 107, 10232-10237.
Medline pdf Supplementary Info
Rickheit G., Wartosch L., Schaffer S., Stobrawa S.M., Novarino G., Weinert S., Jentsch, T.J. (2010).
Role of ClC-5 in renal endocytosis is unique among CLC exchangers and does not require PY-motif-dependent ubiquitylation.
J. Biol Chem. 285, 17595-17603.
Medline pdf Supplementary Info
Preston P., Wartosch L., Günzel D., Fromm M., Kongsuphol P., Ousingsawat J., Kunzelmann K., Barhanin J., Warth R., Jentsch, T.J. (2010).
Disruption of the K+ channel β-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport.
J. Biol Chem. 285, 7165-7175.
Medline pdf Supplementary Info
Wartosch L., Fuhrmann J.C., Schweizer M., Stauber T., Jentsch T.J. (2009).
Lysosomal degradation of endocytosed protein depends on the chloride transport protein ClC-7.
FASEB J. 23, 4056-4068.
Medline pdf Supplementary Info
Bergsdorf E-Y, Zdebik A.A., Jentsch T.J. (2009).
Residues important for nitrate/proton coupling in plant and mammalian CLC transporters.
J. Biol. Chem. 284, 11184-11193.
Medline pdf
Pfeffer C.K., Stein V., Keating D.J., Maier H., Rinke I., Rudhard Y., Hentschke M., Rune G., Jentsch T.J., Hübner C.A. (2009).
NKCC1-dependent GABArgic excitation drives synpatic network maturation during early hippocampal development.
J. Neurosci. 29, 3419-3430.
Medline pdf Supplementary Info (pdf)
Rickheit G., Maier H., Strenzke N., Andreescu C.E., De Zeeuw C.I., Zdebik A.A., Jentsch T.J. (2008).
Endocochlear potential depends on chloride channels: mechanism underlying deafness in Bartter syndrome IV.
EMBO J. 27, 2907-2917.
Medline pdf Supplementary Info (pdf) OMIM
Maritzen T., Keating D.J., Neagoe I., Zdebik A.A., Jentsch T.J. (2008).
Role of the vesicular chloride transporter ClC-3 in neuroendocrine tissue.
J. Neurosci. 28, 10587-10598
Medline pdf Supplementary Info (pdf)
Zdebik A.A., Zifarelli G., Bergsdorf E.-Y., Soliani P., Scheel O., Jentsch T.J., Pusch M. (2008).
Determinants of anion-proton coupling in mammalian endosomal CLC proteins.
J. Biol. Chem. 283, 4219-4227.
Medline pdf Supplementary Info (pdf)
Blanz J., Schweizer M., Auberson M., Maier H., Muenscher A., Hübner C.A., Jentsch T.J. (2007).
Leukoencephalopathy upon disruption of the chloride channel ClC-2.
J. Neurosci. 27, 6581-6589.
Medline, pdf Supplementary Info (pdf)
Rust M.B., Alper S.L., Rudhard Y., Shmukler B.E., Vicente R., Brugnara C., Trudel M., Jentsch T.J., Hübner C.A. (2007).
Disruption of erythroid KCl-cotransporters alters erythrocyte volume and partially rescues erythrocyte dehydration in SAD mice.
J. Clin. Invest. 117, 1708-1717.
Medline, pdf, Supplementary Info (pdf)
Poët M., Kornak U., Schweizer M., Zdebik A.A., Scheel O., Hoelter S., Wurst W., Schmitt A., Fuhrmann J.C., Planells-Cases R., Mole S.E., Hübner C.A., Jentsch T.J. (2006).
Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.
Proc. Natl. Acad. Sci. U S A. 103, 13854-13859.
Medline pdf
Lange P.F., Wartosch L., Jentsch T.J., Fuhrmann J.C. (2006).
ClC-7 requires Ostm1 as a β-subunit to support bone resorption and lysosomal function.
Nature 440, 220-223.
Medline, pdf, Supplementary Info (pdf), OMIM
Kharkovets T., Dedek K., Maier H., Schweizer M., Khimich D., Nouvian R., Vardanyan V., Leuwer R., Moser T., Jentsch T.J. (2006).
Mice with altered KCNQ4 K+ channels implicate sensory outer hair cells in human progressive deafness.
EMBO J. 25, 642-652.
Medline, pdf, Supplementary Info (pdf) OMIM
Rust M.B., Faulhaber J., Budack M.K., Pfeffer C., Maritzen T., Didié M., Beck F.X., Boettger T., Schubert R., Ehmke H., Jentsch T.J., Hübner C.A. (2006).
Neurogenic mechanisms contribute to hypertension in mice with disruption of the K-Cl cotransporter KCC3.
Circ. Res. 98 , 549-556.
Medline, pdf
Scheel O., Zdebik A.A., Lourdel S., Jentsch T.J. (2005).
Voltage-dependent electrogenic chloride-proton exchange by endosomal CLC proteins.
Nature 436, 424-427.
Medline pdf
Schenzer A., Friedrich T., Pusch M., Saftig P, Jentsch T.J., Grötzinger J., Schwake M. (2005).
Molecular determinants of KCNQ (Kv7) K+ channel sensitivity to the anticonvulsant retigabine.
J. Neurosci. 25, 5051-5060.
Medline
Kasper D., Planells-Cases R., Fuhrmann J.C., Scheel O., Zeitz O., Ruether K., Schmitt A., Poët M., Steinfeld R., Schweizer M., Kornak U., Jentsch T.J. (2005).
Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration.
EMBO J. 24, 1079-1091.
Medline, pdf, Supplementary Info (pdf) OMIM
Zdebik A.A., Cuffe J., Bertog M., Korbmacher C., Jentsch T.J. (2004).
Additional disruption of the ClC-2 Cl- channel does not exacerbate the cystic fibrosis phenotype of CFTR mouse models.
J. Biol. Chem. 279, 22276-22283.
Medline pdf
Estévez R., Pusch M., Ferrer-Costa C., Orozco M., Jentsch T.J. (2004).
Functional and structural conservation of CBS domains from CLC channels.
J. Physiol. 557.2, 363-378.
Medline
Boettger T., Rust M.B., Maier H., Seidenbecher T., Schweizer M., Keating D., Faulhaber J., Ehmke H., Pfeffer C., Scheel O., Lemcke B., Host J., Leuwer R., Pape H.C., Völkl H., Hübner C.A., Jentsch T.J. (2003).
Loss of K-Cl cotransporter KCC3 causes deafness, neurodegeneration and reduced seizure threshold.
EMBO J. 22, 5422-5434.
Medline, Supplementary Info (pdf)
Estévez R., Schroeder B.C., Accardi A., Jentsch T.J., Pusch M. (2003).
Conservation of chloride channel structure revealed by an inhibitor binding site in ClC-1.
Neuron 38, 47-59.
Medline, Neuron Preview (pdf)
Schwake M., Jentsch T.J., Friedrich T. (2003).
A carboxy-terminal domain determines the subunit specificity of KCNQ K+ channel assembly.
EMBO Rep. 4, 76-81.
Medline, pdf
Boettger T., Hübner C.A., Maier H., Rust M.B., Beck F.X., Jentsch T.J. (2002).
Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter KCC4.
Nature 416, 874-878.
Medline, Supplementary Info (pdf)
Estévez R., Boettger T., Stein V., Birkenhäger R., Otto E., Hildebrandt F., Jentsch T.J. (2001).
Barttin is a Cl- channel ß-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion.
Nature 414, 558-561.
Medline, News and Views, OMIM, Supplementary Info (pdf)
Dedek K., Kunath B., Kananura C., Reuner U., Jentsch T.J., Steinlein O. (2001)
Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel.
Proc. Natl. Acad. Sci. U.S.A. 98, 12272-12277.
Medline, pdf, OMIM
Betz R.C., Schoser B.G., Kasper D., Ricker K., Ramírez A., Stein V., Torbergsen T., Lee Y.A., Nothen M.M., Wienker T.F., Malin J.P., Propping P., Reis A., Mortier W., Jentsch T.J., Vorgerd M., Kubisch C. (2001)
Mutations in CAV3 cause mechanical hyperirritability of skeletal muscle in rippling muscle disease.
Nature Genet. 28, 218-219.
Medline, OMIM
Hübner C.A., Stein V., Hermans-Borgmeyer I., Meyer T., Ballanyi K., Jentsch T.J. (2001)
Disruption of KCC2 reveals an essential role of K-Cl-cotransport already in early synaptic inhibition.
Neuron 30, 515-524.
Medline
Bösl M.R., Stein V., Hübner C., Zdebik A.A., Jordt S.E., Mukhophadhay A.K., Davidoff M.S., Holstein A.F., Jentsch T.J. (2001)
Male germ cells and photoreceptors, both depending on close cell-cell interactions, degenerate upon ClC-2 Cl--channel disruption.
EMBO J. 20, 1289-1299.
Medline
Kornak U., Kasper D., Bösl M.R., Kaiser E., Schweizer M., Schulz A., Friedrich W., Delling G., Jentsch T.J. (2001)
Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man.
Cell 104, 205-215.
Medline, Nature News & Views, OMIM pdf
Stobrawa S.M., Breiderhoff T., Takamori S., Engel D., Schweizer M., Zdebik A.A., Bösl M.R., Ruether K., Jahn H., Draguhn A., Jahn R., Jentsch T.J. (2001)
Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus.
Neuron 29, 185-196.
Medline, Neuron Preview, Nature Rev Neurosci Highlight
Schwake M., Friedrich T., Jentsch T.J. (2001)
An internalization signal in ClC-5, an endosomal Cl--channel mutated in Dent's disease.
J. Biol. Chem. 276, 12049-12054.
Medline
Weinreich F., Jentsch T.J. (2001)
Pores formed by single subunits in mixed dimers of different CLC chloride channels.
J. Biol. Chem. 276, 2347-2353.
Medline
Piwon N., Günther W., Schwake M., Bösl M.R., Jentsch T.J. (2000)
ClC-5 Cl--channel disruption impairs endocytosis in a mouse model for Dent's disease.
Nature 408, 369-373.
Medline, OMIM, Supplementary Info (Word)
Kornak U., Schulz A., Friedrich W., Uhlhaas S., Kremens B., Voit T., Hasan C., Bode U., Jentsch T.J., Kubisch C. (2000)
Mutations in the a3 subunit of the vacuolar H+-ATPase cause infantile malignant osteopetrosis.
Hum. Mol. Genet. 13, 2059-2063.
Medline, OMIM
Waldegger S., Jentsch T.J. (2000)
Functional and Structural Analysis of ClC-K Chloride Channels Involved in Renal Disease.
J. Biol. Chem. 275, 24257-24533.
Medline
Schroeder B.C., Hechenberger M., Weinreich F., Kubisch C., Jentsch T.J. (2000)
KCNQ5, a Novel Potassium Channel Broadly Expressed in Brain, Mediates M-type Currents.
J. Biol. Chem. 275, 24089-24095.
Medline
Kharkovets T., Hardelin J.-P., Safieddine S., Schweizer M., El-Amraoui A., Petit C., Jentsch T.J. (2000)
KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.
Proc. Natl. Acad. Sci. U.S.A. 97, 4333-4338.
Medline, Commentary
Schroeder B.C., Waldegger S., Fehr S., Bleich M., Warth R., Greger R., Jentsch T.J. (2000)
A constitutively open potassium channel formed by KCNQ1 and KCNE3.
Nature 403, 196-199.
Medline, Supplementary Info (pdf), Comment in TIPS (pdf)
Schriever A.M., Friedrich T., Pusch M., Jentsch T.J. (1999)
CLC chloride channels in Caenorhabditis elegans.
J. Biol. Chem. 274, 34238-34244.
Medline
Friedrich T., Breiderhoff T., Jentsch T.J. (1999)
Mutational analysis demonstrates that ClC-4 and ClC-5 directly mediate plasma membrane currents.
J. Biol. Chem. 274, 896-902.
Medline
Schroeder B. C., Kubisch C., Stein V., Jentsch T. J. (1998)
Moderate loss of function of cyclic-AMP-modulated KCNQ2/KCNQ3 K+ channels causes epilepsy.
Nature 396, 687-690.
Medline
Günther W., Lüchow A., Cluzeaud F., Vandewalle A., Jentsch T.J. (1998)
ClC-5, the chloride channel mutated in Dent's disease, co-localizes with the proton pump in endocytotically active kidney cells.
Proc. Natl. Acad. Sci. U.S.A. 95,8075-8080.
Medline, Commentary pdf
Schwappach B., Stobrawa S., Hechenberger M., Steinmeyer K., Jentsch T.J. (1998)
Golgi localization and functionally important domains in the NH2 and COOH terminus of the yeast ClC putative chloride channel Gef1p.
J. Biol. Chem. 274, 15110-15118.
Medline
Biervert C., Schroeder B.C., Kubisch C., Berkovic S.F., Propping P., Jentsch T.J., Steinlein O.K. (1998)
A potassium channel mutation in neonatal human epilepsy.
Science 279, 403-406.
Medline, OMIM
Jordt S.E., Jentsch T.J. (1997).
Molecular dissection of gating in the ClC-2 chloride channel.
EMBO J. 16, 1582-1592.
Medline
Schmidt-Rose T., Jentsch T.J. (1997).
Transmembrane topology of a ClC chloride channel.
Proc. Natl. Acad. Sci. U.S.A. 94, 7633-7638.
Medline
Ludewig U., Pusch M., Jentsch T.J. (1996).
Two physically distinct pores in the dimeric ClC-0 chloride channel.
Nature 383, 340-343.
Medline, News and Views
Hechenberger M., Schwappach B., Fischer W.N., Frommer W.B., Jentsch T.J., Steinmeyer K. (1996).
A family of putative chloride channels from Arabidopsis and functional complementation of a yeast strain with a ClC gene disruption.
J. Biol. Chem. 271, 33632-33638.
Medline
Lloyd S.E., Pearce S.H.S., Fisher S.E., Steinmeyer K., Schwappach B., Scheinman S.S., Harding B., Bolino M., Devoto M., Goodyer P., Rigden S.P.A., Wrong O., Jentsch T.J., Craig I.W., Thakker R.V. (1996).
A common molecular basis for three inherited kidney stone diseases.
Nature 379, 445-449.
Medline, News and Views, OMIM
Lorenz C., Pusch M., Jentsch T.J. (1996).
Heteromultimeric ClC chloride channels with novel properties.
Proc. Natl. Acad. Sci. U.S.A. 93, 13362-13366.
Medline
Pusch M., Steinmeyer K., Koch M.C., Jentsch T.J. (1995).
Mutations in dominant human myotonia congenita drastically alter the voltage-dependence of the ClC-1 chloride channel.
Neuron 15, 1455-1463.
Medline
Pusch M., Ludewig U., Rehfeldt A., Jentsch T.J. (1995).
Gating of the voltage-dependent chloride channel ClC-0 by the permeant anion.
Nature 373, 527-531.
Medline
Steinmeyer K., Schwappach B., Bens M., Vandewalle A., Jentsch T.J. (1995).
Cloning and functional expression of rat ClC-5, a chloride channel related to kidney disease.
J. Biol. Chem. 270, 31172-31177.
Medline
Kieferle S., Fong P., Bens M., Vandewalle A., Jentsch T.J. (1994).
Two highly homologous members of the ClC chloride channel family in both rat and human kidney.
Proc. Natl. Acad. Sci. U.S.A. 91, 6943-6947.
Medline
Steinmeyer K., Lorenz C., Pusch M., Koch M.C., Jentsch T.J. (1994).
Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen).
EMBO J. 13, 737-743.
Medline
Gründer S., Thiemann A., Pusch M., Jentsch T.J. (1992).
Regions involved in the opening of ClC-2 chloride channel by voltage and cell volume.
Nature 360, 759-762.
Medline pdf
Koch M.C., Steinmeyer K., Lorenz C., Ricker K., Wolf F., Otto M., Zoll B., Lehmann-Horn F., Grzeschik K.H., Jentsch T.J. (1992).
The skeletal muscle chloride channel in dominant and recessive human myotonia.
Science 257, 797-800.
Medline pdf
Thiemann A., Gründer S., Pusch M., Jentsch T.J. (1992).
A chloride channel widely expressed in epithelial and non-epithelial cells.
Nature 356, 57-60.
Medline pdf
Bauer C.K., Steinmeyer K., Schwarz J.R., Jentsch T.J. (1991).
Completely functional double-barreled chloride channel expressed from a single Torpedo cDNA.
Proc. Natl. Acad. Sci. U.S.A. 88, 11052-11056.
Medline
Steinmeyer K., Klocke R., Ortland C., Gronemeier M., Jockusch H., Gründer S., Jentsch T.J. (1991).
Inactivation of muscle chloride channel by transposon insertion in myotonic mice.
Nature 354, 304-308.
Medline pdf
Steinmeyer K., Ortland C., Jentsch T.J. (1991).
Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel.
Nature 354, 301-304.
Medline pdf
Jentsch T.J., Steinmeyer K., Schwarz, G. (1990).
Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes.
Nature 348, 510-514.
Medline, News and Views pdf
Jentsch T.J., Garcia A.M., Lodish H.F. (1989).
Primary structure of a novel 4-acetamido-4'- isothiocyanostilbene-2,2'-disulphonic acid (SITS)-binding membrane protein highly expressed in Torpedo electroplax.
Biochem. J. 261, 155-166.
Medline
Jentsch T.J., Janicke I., Sorgenfrei D., Keller S.K., Wiederholt M. (1986).
The regulation of intracellular pH in monkey kidney epithelial cells (BSC-1). Role of Na+/H+-antiport, Na+-HCO3-(NaCO3-) symport, and Cl-/HCO3- exchange.
J. Biol. Chem. 261, 12120-12127.
Medline
Jentsch T.J., Keller S.K., Koch M., Wiederholt M. (1984).
Evidence for coupled transport of bicarbonate and sodium in cultured bovine corneal endothelial cells.
J. Membrane Biol. 81, 189-204.
Medline pdf
Ernst N., Jentsch T. (1981).
Post-field ionization of singly charged rhodium: An experimental and theoretical study.
Phys. Rev. B 24, 6234-6240.
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