Old publications

Papers and reviews (for recent publications see here):


Zifarelli G, Pusch M. 2015. CLC chloride channels and transporters. In Handbook of Ion Channels. doi: 10.1201/b18027-35 (J. Zheng, M.C. Trudeau eds.)

Barrallo-Gimeno A, Gradogna A, Zanardi I, Pusch M, Estévez R. 2015. Regulatory/Auxiliary subunits of CLC chloride channel/transport proteins. J Physiol doi: 10.1113/JP270057

Gründer S, Pusch M. 2015. Biophysical properties of acid-sensing ion channels (ASICs). Neuropharmacology doi: 10.1016/j.neuropharm.2014.12.016

Soattin L, Fiore M, Gavazzo P, Viti F, Facci P, Raiteri R, Difato F, Pusch M, Vassalli M. 2015. The biophysics of piezo1 and piezo2 mechanosensitive channels. Biophys Chem S0301-4622(15)30018-1. doi: 10.1016/j.bpc.2015.06.013

Pusch M, Zifarelli G. 2014. ClC-5: Physiological role and biophysical mechanisms. Cell Calcium doi: 10.1016/j.ceca.2014.09.007

Pusch M, Zifarelli G. 2014. Thermal sensitivity of CLC and TMEM16 chloride channels and transporters. Curr Top Membr 74:213-231

Zifarelli G, Pusch M. 2013. CLC channels and transporters. In Encyclopedia of Biophysics. doi: 10.1007/978-3-642-16712-6_360 (G.C.K. Roberts Editor)

Zifarelli G, Pusch M. 2012. A kick-start for CLC antiporters' pharmacology. Chem Biol 19:1358-1359

Gradogna A, Fenollar-Ferrer C, Forrest LR, Pusch M. 2012. Dissecting a regulatory calcium-binding site of CLC-K kidney chloride channels. J Gen Physiol 140:681-696

Costa A, Gutla PV, Boccaccio A, Scholz-Starke J, Festa M, Basso B, Zanardi I, Pusch M, Schiavo FL, Gambale F, Carpaneto A. 2012. The Arabidopsis central vacuole as an expression system for intracellular transporters: functional characterization of the Cl-/H+ exchanger CLC-7. J Physiol 590:3421-3430

Zifarelli G, De Stefano S, Zanardi I, Pusch M. 2012. On the mechanism of gating charge movement of ClC-5, a human Cl/H+ antiporter. Biophys J 102:2060-2069

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. 2012. GlialCAM, a Protein Defective in a Leukodystrophy, Serves as a ClC-2 Cl(-) Channel Auxiliary Subunit. Neuron 73:951-961

Ruivo R, Bellenchi GC, Chen X, Zifarelli G, Sagné C, Debacker C, Pusch M, Supplisson S, Gasnier B. 2012. Mechanism of proton/substrate coupling in the heptahelical lysosomal transporter cystinosin. Proc Natl Acad Sci U S A. 109(5):E210-E217

De Stefano S, Pusch M, Zifarelli G. 2011. Extracellular determinants of anion discrimination of the Cl-/H+ antiporter protein CLC-5. J Biol Chem 286:44134-44144

Strutz-Seebohm N, Pusch M, Wolf S, Stoll R, Tapken D, Gerwert K, Attali B, Seebohm G. 2011. Structural basis of slow activation gating in the cardiac I Ks channel complex. Cell Physiol Biochem

Gradogna A, Babini E, Picollo A, Pusch M. 2010. A regulatory calcium-binding site at the subunit interface of CLC-K kidney chloride channels. J Gen Physiol 136:311-323

Zifarelli G, Liantonio A, Gradogna A, Picollo A, Gramegna G, De Bellis M, Murgia AR, Babini E, Camerino DC, Pusch M. 2010. Identification of sites responsible for the potentiating effect of niflumic acid on ClC-Ka kidney chloride channels. Brit J Pharmacol. 160: 1652-1661

Pangrazio A, Pusch M, Caldana E, Frattini A, Lanino E, Tamhankar PM, Phadke S, Lopez AG, Orchard P, Mihci E, Abinun M, Wright M, Vettenranta K, Bariae I, Melis D, Tezcan I, Baumann C, Locatelli F, Zecca M, Horwitz E, Mansour LS, Van Roij M, Vezzoni P, Villa A, Sobacchi C. 2010. Molecular and clinical heterogeneity in CLCN7-dependent osteopetrosis: report of 20 novel mutations. Human Mutat. 31:E1071-E1080

Zifarelli G, Pusch M. 2010. Relaxing messages from the sarcolemma. J Gen Physiol 136:593-596

Gradogna A, Pusch M. 2010. Molecular pharmacology of kidney and inner ear CLC-K chloride channels. Frontiers in Pharmacology 1:130

Zifarelli G, Pusch M. 2010. CLC transport proteins in plants. FEBS Lett. 584:2122-2127

Zifarelli G, Pusch M. 2009. Intracellular regulation of human ClC-5 by adenine nucleotides. EMBO Rep. 10:1111-1116

Zifarelli G, Pusch M. 2009. Conversion of the 2 Cl(-)/1 H(+) antiporter ClC-5 in a NO(3)(-)/H(+) antiporter by a single point mutation. EMBO J. 28:175-182

Pusch M, Zifarelli G. 2009. It's the proton also in ClC-2. J Physiol 587:1379-1380

Zifarelli G, Pusch M. 2009. The role of protons in fast and slow gating of the Torpedo chloride channel ClC-0. Eur Biophys J 2009 Jan 9.

Zdebik AA, Zifarelli G, Bergsdorf E-Y, Soliani P, Scheel O, Jentsch TJ, Pusch M. 2008. Determinants of anion-proton coupling in mammalian endosomal CLC proteins. J Biol Chem  283:4219-4227

Zifarelli G, Soliani P, Pusch M. 2008. Buffered diffusion around a spherical proton pumping cell: a theoretical analysis. Biophys J 94: 53-62

Zifarelli G, Murgia AR, Soliani P, Pusch M. 2008. Intracellular proton regulation of ClC-0. J Gen Physiol. 132:185-198

Kahlig KM, Rhodes TH, Pusch M, Freilinger T, Pereira-Monteiro JM, Ferrari MD, van den Maagdenberg AM, Dichgans M, George AL Jr. 2008. Divergent sodium channel defects in familial hemiplegic migraine. Proc Natl Acad Sci U S A. 105:9799-9804

Liantonio A, Picollo A, Carbonara G, Fracchiolla G, Tortorella P, Loiodice F, Laghezza A, Babini E, Zifarelli G, Pusch M, Camerino DC. 2008. Molecular switch for CLC-K Cl- channel block/activation: optimal pharmacophoric requirements towards high-affinity ligands. Proc Natl Acad Sci U S A. 105:1369-1373

Zifarelli G, Pusch M. 2008. The muscle chloride channel ClC-1 is not directly regulated by intracellular ATP. J Gen Physiol 131:109-116

de Vries B, Freilinger T, Vanmolkot KRJ, Koenderink JB, Stam AH, Terwindt GM, Babini E, van den Boogerd EH, van den Heuvel JJMW, Frants RR, Haan J, Pusch M, van den Maagdenberg AMJM, Ferrari MD, Dichgans M. 2007. Systematic analysis of three FHM genes in 39 sporadic patients with hemiplegic migraine. Neurology 69:2170-2176.

Picollo A, Liantonio A, Babini E, Conte Camerino D,  Pusch M. 2007. Mechanism of interaction of niflumic acid with heterologously expressed kidney CLC-K chloride channels. J Membr Biol 216: 73-82

Vanmolkot KR, Babini E, de Vries B, Stam AH, Freilinger T, Terwindt GM, Norris L, Haan J, Frants RR, Ramadan NM, Ferrari MD, Pusch M, van den Maagdenberg AM, Dichgans M. 2007. The novel p.L1649Q mutation in the SCN1A epilepsy gene is associated with familial hemiplegic migraine: genetic and functional studies. Hum Mutat. 28:522.

Liantonio A, Giannuzzi V, Picollo A, Babini E, Pusch M, Conte Camerino D. 2007. Niflumic acid inhibits chloride conductance of rat skeletal muscle by directly inhibiting the CLC-1 channel and by increasing intracellular calcium. Br J Pharmacol. 150:235-47.

Macias MJ, Teijido O, Zifarelli G, Martin P, Ramirez-Espain X, Zorzano A, Palacin M, Pusch M, Estevez R. 2007. Myotonia-related mutations in the distal C-terminus of ClC-1 and ClC-0 chloride channels affect the structure of a poly-proline helix. Biochem J 403:79-87.

Zifarelli G, Pusch M. 2007. CLC chloride channels and transporters: a biophysical and physiological perspective. Rev Physiol Biochem Pharmacol 158:23-76.

Pusch M. 2007. Chloride transport across biological membranes, vol 38. Elsevier, Amsterdam, pp 83-108.

Pusch M, Liantonio A, De Luca A, Conte Camerino D. 2007. Pharmacology of CLC chloride channels and transporters. In: Pusch M (ed) Chloride transport across biological membranes, vol 38. Elsevier, Amsterdam, pp 83-108.

Traverso S, Zifarelli G, Aiello R, Pusch M. 2006. Proton sensing of CLC-0 mutant E166D. J Gen Physiol 127: 51-65.

Liantonio A, Picollo A, Babini E, Carbonara G, Fracchiolla G, Loiodice F, Tortorella V, Pusch M, Camerino DC. 2006. Activation and inhibition of kidney CLC-K chloride channels by fenamates. Mol Pharmacol 69: 165-173.

Chen X, Paukert M, Kadurin I, Pusch M, Gründer S. 2006. Strong modulation by RFamide neuropeptides of the ASIC1b/3 heteromer in competition with extracellular calcium. Neuropharmacology, 50, 964-974.

Pusch M, Zifarelli G, Murgia AR, Picollo A, Babini E. 2006. Channel or transporter? The CLC saga continues. Exp Physiol, 91, 149-152. pdf.

Pusch M. 2006. Chloride transporting CLC proteins. In: Biological Membrane Ion Channels: Dynamics, Structure, and Applications (eds. S. H. Chung, O. S. Andersen and V. Krishnamurthy). Springer, New York, Chapter 8, Pp. 301-333.

Pusch M, Jentsch TJ. 2005. Unique structure and function of chloride transporting CLC proteins. IEEE Trans Nanobioscience 4:49-57. pdf.

Picollo A, Pusch M.  2005. Chloride / proton antiporter activity of mammalian CLC proteins ClC-4 and ClC-5. Nature, 436, 420-423.

Schenzer A, Friedrich T, Pusch M, Saftig P, Jentsch TJ, Grötzinger J, Schwake M. 2005. Molecular determinants of KCNQ K+ channel sensitivity to the anticonvulsant retigabine. J Neurosc 25, 5051-5060.

Dichgans M, Freilinger T,  Eckstein G, Babini E, Lorenz-Depiereux  B, Biskup S, Ferrari MD, Herzog J, van den Maagdenberg  AMJM, Pusch M, Strom TM. 2005. Mutation in the neuronal voltage-gated sodium channel SCN1A causes familial hemiplegic migraine. Lancet, 366, 371-377.

Teijido O, Martinez A, Pusch M, Zorzano A, Soriano E, Del Rio JA, Palacin M, Estévez R. 2004. Localization and functional analyses of the MLC1 protein involved in  megalencephalic leukoencephalopathy with subcortical cysts. Hum Mol Genet 13, 2581-2594.

Paukert M, Babini E, Pusch M, Gründer, S. 2004. Identification of the Ca2+ Blocking Site of Acid-sensing Ion Channel (ASIC) 1: Implications for Channel Gating. J Gen Physiol 124, 383-394.

Pusch M. 2004. Ca(2+)-activated chloride channels go molecular. J Gen Physiol 123:323-325.

Estévez R, Pusch M, Ferrer-Costa C, Orozco M, Jentsch TJ. 2004. Functional and structural conservation of CBS domains from CLC chloride channels. J Physiol 557:363-378.

Conte Camerino D, Tricarico D, Pierno S, Desaphy JF, Liantonio A, Pusch M, Burdi R, Camerino C, Fraysse B, De Luca A. 2004. Taurine and skeletal muscle disorders. Neurochem Res 29:135-142.

Picollo A, Liantonio A, Didonna MP, Elia L, Camerino DC, Pusch M. 2004. Molecular determinants of differential pore blocking of kidney CLC-K chloride channels. EMBO Rep 5:584-589.

Liantonio A, Pusch M, Picollo A, Guida P, De Luca A, Pierno S, Fracchiolla G, Loiodice F, Tortorella P, Conte Camerino D. 2004. Investigations of pharmacological properties of the renal CLC-K1 chloride channel co-expressed with barttin by the use of 2-(p-chlorophenoxy) propionic acid derivatives and other structurally unrelated chloride channels blockers. J Am Soc Nephrol 15:13-20.

Babini E, Pusch M. 2004. A two-holed story: structural secrets about ClC proteins become unraveled? Physiology 19:293-299. pdf.

Pusch M. 2004. Structural insights into chloride and proton mediated gating of CLC chloride channels. Biochemistry (USA) 43:1135-1144. pdf.

Seebohm G, Pusch M, Chen J, Sanguinetti MC. 2003. Pharmacological activation of normal and arrhythmia-associated mutant KCNQ1 potassium channels. Circ Res 23, 941-7.

Traverso S, Elia L, Pusch M. 2003. Gating competence of constitutively open CLC-0 mutants revealed by the interaction with a small organic Inhibitor. J Gen Physiol 122, 295-306.

Accardi A, Pusch M. 2003. Conformational changes in the pore of CLC-0. J Gen Physiol 122, 277-293.

Liantonio A, De Luca A, Pierno S, Didonna MP, Loiodice F, Fracchiolla G, Tortorella P, Antonio L, Bonerba E, Traverso S, Elia L, Picollo A, Pusch M, Camerino DC. 2003. Structural requisites of 2-(p-chlorophenoxy)propionic acid analogues for activity on native rat skeletal muscle chloride conductance and on heterologously expressed CLC-1. Br J Pharmacol 139:1255-1264.

Seebohm G, Sanguinetti MC, Pusch M. 2003. Tight coupling of Rubidium conductance and inactivation  in human KCNQ1 potassium channels. J Physiol (Lond) 552, 369-78.

Moran O, Traverso S, Elia L, Pusch M. 2003. Molecular modeling of p-chlorophenoxyacetic acid binding to the CLC-0 channel. Biochemistry 42, 5176-5185.

Estévez R, Schroeder BC, Accardi A, Jentsch TJ, Pusch M. 2003. Conservation of chloride channel structure revealed by an inhibitor binding site in ClC-1. Neuron 38, 47-59.

Liantonio A, Accardi A, Carbonara G, Fracchiolla G, Loiodice F, Tortorella P, Traverso S, Guida P, Pierno S, De Luca A, Camerino D C, Pusch M. Molecular requisites for drug binding to muscle CLC-1 and renal CLC-K channel revealed by the use of phenoxy-alkyl derivatives of 2-(p-chlorophenoxy)propionic acid. 2002. Mol Pharmacol 62:265-271.

Pusch M, Accardi A, Liantonio A, Guida P, Traverso S, Camerino DC, Conti F. 2002. Mechanisms of block of muscle type CLC chloride channels (Review). Mol Membr Biol 19:285-92.

Pusch M. 2002. Myotonia caused by mutations in the muscle chloride channel gene CLCN1. Hum Mut 19: 423-434.

Seebohm G, Lerche C, Pusch M, Steinmeyer K, Bruggemann A, Busch AE. 2001. A kinetic study on the stereospecific inhibition of KCNQ1 and I(Ks) by the chromanol 293B. Br J Pharmacol 134:1647-1654.

Accardi A, Ferrera L, Pusch M. 2001. Drastic  reduction of the slow gate of human ClC-1 by mutation C277S. J Physiol (Lond) 534:745-752.

Pusch M, Accardi A, Liantonio A, Ferrera L, De Luca A, Camerino DC, Conti F. 2001. Mechanism of block of single protopores of the Torpedo chloride channel CLC-0 by 2-(p-chlorophenoxy)butyric acid (CPB). J Gen Physiol 118:45-62.

Pusch M, Ferrera L, Friedrich T. 2001. Two open states and rate-limiting gating steps revealed by intracellular Na+ block of human KCNQ1 and KCNQ1/KCNE1 K+ channels. J Physiol (Lond) 533:135-43.

Rychkov G, Pusch M, Roberts M, Bretag A. 2001. Interaction of hydrophobic anions with the rat skeletal muscle chloride channel ClC-1: effects on permeation and gating. J Physiol (Lond) 530:379-93.

Pusch M. 2001. Chloride channelopathies. Pharmaceutical News 8:45-51.

Pusch M. 2000. Negative thinking: anion channels. Review of "Chloride channels", edited by R. Kozlowski (Iris Media Limited, Feb. 2000), Nature Cell Biol 2:E150.

Accardi A, Pusch M. 2000. Fast and slow gating relaxations in the muscle chloride channel CLC-1. J Gen Physiol 116:433-444.

Pusch M, Liantonio A, Bertorello L, Accardi A, De Luca A, Pierno S, Tortorella V, Conte Camerino D. 2000. Pharmacological characterization of chloride channels belonging to the ClC family by the use of chiral clofibric acid derivatives. Mol Pharmacol 58:498-507.

Nobile M, Pusch M, Rapisarda C, Ferroni S. 2000. Single-channel analysis of a ClC-2-like chloride conductance in cultured rat cortical astrocytes. FEBS Lett 479:10-14.

Schwake M, Pusch M, Kharkovets T, Jentsch TJ. 2000. Surface expression and single channel properties of KCNQ2/KCNQ3, M-type K+ channels involved in epilepsy. J Biol Chem 275:13343-13348.

Pusch M, Bertorello L, Conti F. 2000. Gating and flickery block differentially affected by rubidium in homomeric KCNQ1 and heteromeric KCNQ1/KCNE1 potassium channels. Biophys J 78:211-226.

Schriever AM, Friedrich T, Pusch M, Jentsch TJ. 1999.  CLC chloride channels in  Caenorhabditis elegans.  J Biol Chem 274:34238-34244.

Saviane C, Conti F, Pusch M. 1999. The muscle chloride channel ClC-1 has a double-barreled appearance that is differentially affected in dominant and recessive myotonia. J Gen Physiol 113:457-467.

Pusch M, Jordt SE, Stein V, Jentsch TJ. 1999. Chloride dependence of hyperpolarization-activated chloride channel gates. J Physiol (Lond) 515:341-353.

Pusch M. 1998. Increase of the single-channel conductance of KvLQT1 potassium channels induced by the association with minK. Pflügers Arch Eur J Physiol 437:172-174.

Pusch M, Magrassi R, Wollnik B, Conti F. 1998. Activation and inactivation of homomeric KvLQT1 potassium channels. Biophys J 75:785-792.

Rychkov GY, Pusch M, Roberts ML, Jentsch TJ, Bretag AH. 1998. Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions. J Gen Physiol 111:653-665.

Ludewig U, Jentsch TJ, Pusch M. 1997. Inward rectification in CLC-0 chloride channels caused by mutations in several protein regions. J Gen Physiol 110:165-171.

Ludewig U, Pusch M, Jentsch TJ. 1997. Independent gating of single pores in CLC-0 chloride channels. Biophys J 73:789-797.

Wollnik B, Kubisch C, Steinmeyer K, Pusch M.1997. Identification of functionally important regions of the muscular chloride channel ClC-1 by analysis of recessive and dominant myotonic mutations. Hum Mol Genet 6:805-811.

Pusch M, Ludewig U, Jentsch TJ. 1997. Temperature dependence of fast and slow gating relaxations of ClC-0 chloride channels. J Gen Physiol 109:105-116.

Ludewig U, Jentsch TJ, Pusch M. 1997. Analysis of a protein region involved in permeation and gating of the voltage-gated chloride channel ClC-0. J Physiol (Lond)  498:691-702.

Jentsch TJ, Pusch M, Schwappach B, Steinmeyer K. 1997.  Myotonia and other diseases due to chloride channels mutations. Nova Acta Leopoldina NF 75, Nr. 302, 75-81.

Lorenz C, Pusch M, Jentsch TJ. 1996. Heteromeric ClC channels with novel properties. Proc Natl Acad Science 93:133262-13366.

Rychkov GY, Pusch M, Astill DStJ, Roberts ML, Jentsch TJ, Bretag AH. 1996. Concentration and pH dependence of skeletal muscle chloride channel ClC-1. J Physiol (Lond) 497:423-435.

Pusch M. 1996. Knocking on channels door: the permeating chloride ion acts as the gating charge in ClC-0. J Gen Physiol 108:233-236.

Ludewig U, Pusch M, Jentsch TJ. 1996. Two physically distinct pores in the dimeric ClC-0 chloride channel. Nature 382:340-343.

Conti F, Gheri A, Pusch M, Moran O. 1996. Use-dependence of TTX block of sodium channels. A Revival of the trapped-ion mechanism. Biophys J 71:1295-1312.

Pusch M, Steinmeyer K, Koch MC, Jentsch TJ. 1995. Mutations in dominant human myotonia congenita drastically alter the voltage-dependence of the ClC-1 chloride channel.  Neuron 15:1455-1463.

Pusch M, Ludewig U, Rehfeldt A, Jentsch TJ. 1995. Gating of the voltage dependent chloride channel ClC-0 by the permeant anion. Nature 373:527-530.

Jentsch TJ, Günther W, Pusch M, Schwappach B. 1995. Properties of voltage-gated chloride channels of the ClC gene family. J Physiol (Lond) 482:19S-25S.

Jentsch TJ, Lorenz C, Pusch M, Steinmeyer K. 1995. Myotonias due to ClC-1 chloride channel mutations. In Ion channels and genetic diseases, Vol. 50 of Society of general physiologist series, Rockefeller University press, p. 149-159.

Pusch M, Jentsch TJ. 1994. Molecular physiology of voltage-gated chloride channels. Physiological Reviews 74:813-827.

Pusch M, Steinmeyer K, Jentsch TJ. 1994. Low single channel conductance of the major skeletal muscle chloride channel, ClC-1. Biophys J 66:149-152.

Steinmeyer K, Pusch M, Koch MC, Jentsch TJ. 1994. Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen). EMBO J 13:737-743.

Jentsch TJ, Pusch M, Rehfeldt A, Steinmeyer K. 1993. The ClC-family of voltage-gated chloride channels: structure and function. In Molecular basis of ion channels and receptors involved in nerve excitation, synaptic transmission and muscle contraction, Vol. 707 of the Ann. N.Y. Acad. Science, p. 285-293.

Gründer S, Thiemann A, Pusch M, Jentsch TJ. 1992. Regions involved in the opening of ClC-2 chloride channel by voltage and cell volume. Nature 360:759-762.

Thiemann A, Gründer S, Pusch M, Jentsch TJ. 1992. A chloride channel widely expressed in epithelial and non-epithelial cells. Nature 356:57-60.

Terlau H, Heinemann SH, Stühmer W, Pusch M, Conti F, Imoto K, Numa S. 1991. Mapping the site of block by tetrodotoxin and saxitoxin of sodium channel II. FEBS Lett 293:93-96.

Pusch M, Noda M, Stühmer W, Numa S, Conti F. 1991. Single point mutations of the sodium channel drastically reduce the pore permeability without preventing its gating. Eur. Biophys J 20:127-133. pdf.

Pusch M. 1990. Divalent cations as probes for structure-function relationships of cloned voltage-dependent sodium channels. Eur Biophys J 18:327-333.

Pusch M. 1990. Open channel block of sodium channels by intracellular magnesium. Eur Biophys J 18:317-326.

Pusch M, Conti F, Stühmer W. 1989. Intracellular magnesium blocks sodium outward currents in a voltage- and dose-dependent manner. Biophys J 55:1267-1271.
 

Pusch M, Neher E. 1988. Rates of diffusional exchange between small cells and a measuring patch pipette. Pflügers Arch 411:204-211. pdf.

Penner R, Pusch M, Neher E. 1987. Washout phenomena in dialyzed mast cells allow  discrimination of different steps in stimulus-secretion coupling. Bioscience Reports 7:313-320.

Jansen G, Pusch M, Soff G. 1988. Continuum solutions of the Klein Gordon equation. Z Phys D 8:315-327.