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.
Medlinepdf  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.
MedlineSupplementary 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

Kubisch C., Schroeder B.C., Friedrich T., Lütjohann B., El-Amraoui A., Marlin S., Petit C., Jentsch T.J. (1999)
KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness.
Cell 96, 437-446.
Medline, OMIM    pdf

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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