Genomes and Genes
Summary: Cell membrane glycoproteins selective for chloride ions.
Publications302 found, 100 shown here
- Fipronil is a potent open channel blocker of glutamate-activated chloride channels in cockroach neuronsXilong Zhao
Department of Molecular Pharmacology and Biological Chemistry, Northwestern University Medical School, Chicago, Illinois 60611, USA
J Pharmacol Exp Ther 310:192-201. 2004..and mammals and activity against dieldrin-resistant insects, we studied fipronil action on glutamate-gated chloride channels (GluCls), unique invertebrate ligand-gated chloride channels, in cockroach thoracic ganglion neurons, using ..
- Structure of a eukaryotic CLC transporter defines an intermediate state in the transport cycleLiang Feng
Laboratory of Molecular Neurobiology and Biophysics, Rockefeller University, Howard Hughes Medical Institute, 1230 York Avenue, New York, NY 10065, USA
Science 330:635-41. 2010..Comparison with prokaryotic CLC shows that a gating glutamate residue changes conformation and suggests a basis for 2:1 Cl(-)/H(+) exchange and a simple mechanistic connection between CLC channels and transporters...
- TMEM16A, a membrane protein associated with calcium-dependent chloride channel activityAntonella Caputo
Laboratorio di Genetica Molecolare, Istituto Giannina Gaslini, Genova 16148, Italy
Science 322:590-4. 2008Calcium-dependent chloride channels are required for normal electrolyte and fluid secretion, olfactory perception, and neuronal and smooth muscle excitability. The molecular identity of these membrane proteins is still unclear...
- The spectrum of ocular phenotypes caused by mutations in the BEST1 geneCamiel J F Boon
Department of Ophthalmology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
Prog Retin Eye Res 28:187-205. 2009..The genotype-phenotype correlations that are observed in association with BEST1 mutations are discussed. In addition, in vitro studies and animal models that clarify the pathophysiological mechanisms are reviewed...
- Chloride channels: often enigmatic, rarely predictableCharity Duran
Department of Cell Biology, Emory University School of Medicine, Atlanta, GA 30322, USA
Annu Rev Physiol 72:95-121. 2010....
- Expression cloning of TMEM16A as a calcium-activated chloride channel subunitBjörn Christian Schroeder
Department of Physiology, Howard Hughes Medical Institute, University of California San Francisco, San Francisco, CA 94143, USA
Cell 134:1019-29. 2008Calcium-activated chloride channels (CaCCs) are major regulators of sensory transduction, epithelial secretion, and smooth muscle contraction...
- Pathophysiology and puzzles of the volume-sensitive outwardly rectifying anion channelYasunobu Okada
Department of Cell Physiology, National Institute for Physiological Sciences, Myodaiji cho, Okazaki 444 8585, Japan
J Physiol 587:2141-9. 2009..Molecular identification of VSOR is required to address the question of how cells sense volume expansion and activate VSOR...
- Functional and clinical data of Best vitelliform macular dystrophy patients with mutations in the BEST1 geneGiuseppe Querques
Department of Ophthalmology, Hopital Intercommunal de Creteil, University Paris XII, Paris, France
Mol Vis 15:2960-72. 2009..To analyze functional and clinical data of Best vitelliform macular dystrophy (VMD) patients with mutations in the BEST1 gene...
- Principles of activation and permeation in an anion-selective Cys-loop receptorRyan E Hibbs
Vollum Institute, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, Oregon 97239, USA
Nature 474:54-60. 2011..GluCl provides a framework for understanding mechanisms of fast inhibitory neurotransmission and allosteric modulation of Cys-loop receptors...
- Molecular physiology of bestrophins: multifunctional membrane proteins linked to best disease and other retinopathiesH Criss Hartzell
Department of Cell Biology, Center for Neurodegenerative Disease, Emory University School of Medicine, Atlanta, Georgia 30322, USA
Physiol Rev 88:639-72. 2008..Bestrophins are also regulated by cell volume and may be a member of the volume-regulated anion channel family...
- TMEM16A confers receptor-activated calcium-dependent chloride conductanceYoung Duk Yang
Sensory Research Center, CRI, College of Pharmacy, Seoul National University, Seoul 151 742, Korea
Nature 455:1210-5. 2008Calcium (Ca(2+))-activated chloride channels are fundamental mediators in numerous physiological processes including transepithelial secretion, cardiac and neuronal excitation, sensory transduction, smooth muscle contraction and ..
- Missense mutations in a retinal pigment epithelium protein, bestrophin-1, cause retinitis pigmentosaAlice E Davidson
Genetic Medicine, The University of Manchester, Manchester Academic Heath Science Centre, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK
Am J Hum Genet 85:581-92. 2009..L140V and p.D228N) caused mislocalization of bestrophin-1 from the basolateral membrane to the cytoplasm. Mutations in bestrophin-1 are increasingly recognized as an important cause of inherited retinal dystrophy...
- Absence of the skeletal muscle sarcolemma chloride channel ClC-1 in myotonic miceC A Gurnett
Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa, College of Medicine, Iowa City 52242, USA
J Biol Chem 270:9035-8. 1995..Immunohistochemistry of skeletal muscle from ADR and other mouse models of human muscle disease demonstrate that the absence of ClC-1 chloride channel is a defect specific to ADR mice...
- Mutations in the VMD2 gene are associated with juvenile-onset vitelliform macular dystrophy (Best disease) and adult vitelliform macular dystrophy but not age-related macular degenerationF Kramer
Institut fur Humangenetik, Universitat Wurzburg, Germany
Eur J Hum Genet 8:286-92. 2000..There were no mutations found in the AMD group. In addition, four frequent intragenic polymorphisms did not reveal allelic association of the VMD2 locus with AMD. These data exclude a direct role of VMD2 in the predisposition to AMD...
- Variant phenotype of Best vitelliform macular dystrophy associated with compound heterozygous mutations in VMD2Patrik Schatz
Department of Ophthalmology, Lund University Hospital, Lund, Sweden
Ophthalmic Genet 27:51-6. 2006..To characterize the phenotype of members of a Swedish family with Best macular dystrophy and two distinct mutations in VMD2...
- Residues important for nitrate/proton coupling in plant and mammalian CLC transportersEun Yeong Bergsdorf
Department of Physiology and Pathology of Ion Transport, Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, D 13125 Berlin, Germany
J Biol Chem 284:11184-93. 2009Members of the CLC gene family either function as chloride channels or as anion/proton exchangers. The plant AtClC-a uses the pH gradient across the vacuolar membrane to accumulate the nutrient NO(3)(-) in this organelle...
- Autosomal recessive vitelliform macular dystrophy in a large cohort of vitelliform macular dystrophy patientsTyson R Kinnick
Department of Ophthalmology and Visual Sciences, Carver Family Center for Macular Degeneration, University of Iowa, Iowa City, Iowa 52242, USA
Retina 31:581-95. 2011..To report 11 cases of autosomal recessive vitelliform macular dystrophy and to compare their molecular findings and phenotypic characteristics with those of patients with the more common and well-described dominant form of the disease...
- Detailed analysis of retinal function and morphology in a patient with autosomal recessive bestrophinopathy (ARB)Christina Gerth
Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Doc Ophthalmol 118:239-46. 2009..For the first time, high-resolution imaging provided in vivo evidence of RPE and photoreceptor involvement in ARB...
- Expression profile and protein translation of TMEM16A in murine smooth muscleAlison J Davis
Division of Biomedical Sciences, St George s, University of London, London, United Kingdom
Am J Physiol Cell Physiol 299:C948-59. 2010..This study shows that TMEM16A expression is robust in murine vascular smooth muscle cells, consolidating the view that this gene is a viable candidate for the native Ca(2+)-activated Cl(-) channel in this cell type...
- Novel and homozygous BEST1 mutations in Chinese patients with Best vitelliform macular dystrophyRaymond L M Wong
Department of Ophthalmology and Visual Sciences, Chinese University of Hong Kong, Kowloon, Hong Kong SAR, China
Retina 30:820-7. 2010..The purpose of this study was to investigate the BEST1 gene mutations in Chinese patients with Best vitelliform macular dystrophy (BVMD)...
- Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild type, G551D, and F508del cystic fibrosis transmembrane conductance regulator chloride channelsSabrina Noel
IPBC CNRS UMR 6187, , 40 Avenue du Recteur Pineau, 86022 Poitiers, France
J Pharmacol Exp Ther 319:349-59. 2006..The potency of these agents indicates that compounds in this class may be of therapeutic benefit in CFTR-related diseases, including cystic fibrosis...
- Clinical expression of Best's vitelliform macular dystrophy in Swedish families with mutations in the bestrophin geneV Ponjavic
Department of Ophthalmology, University Hospital, Lund, Sweden
Ophthalmic Genet 20:251-7. 1999..To examine the clinical phenotype of three Swedish families with Best's vitelliform macular dystrophy (BMD) and three different mutations in the recently identified bestrophin gene...
- An ivermectin-sensitive glutamate-gated chloride channel from the parasitic nematode Haemonchus contortusSamantha McCavera
Dept of Biology and Biochemistry, University of Bath, UK
Mol Pharmacol 75:1347-55. 2009Nematode glutamate-gated chloride channels are targets of the macrocyclic lactones, the most important group of anthelmintics available...
- A normal electro-oculography in a family affected by best disease with a novel spontaneous mutation of the BEST1 geneF Testa
Department of Ophthalmology, Second University of Naples, Naples, Italy
Br J Ophthalmol 92:1467-70. 2008..To describe clinical and genetic findings in an Italian family affected by Best disease...
- Differential regulation of Ca(2+)-activated Cl(-) currents in rabbit arterial and portal vein smooth muscle cells by Ca(2+)-calmodulin-dependent kinaseI A Greenwood
Department of Pharmacology and Clinical Pharmacology, St George s Hospital Medical School, London, UK
J Physiol 534:395-408. 2001..5. The present study shows that regulation of Ca(2+)-dependent Cl(-) channels by CaMKII differs between arterial and portal vein myocytes...
- Role of chloride channels in regulating the volume of acinar cells of the rabbit superior lacrimal glandGeorge H Herok
Co operative Research Centre for Eye Research and Technology, University of New South Wales, Kensington, NSW, Australia
Invest Ophthalmol Vis Sci 49:5517-25. 2008..To characterize the outward chloride currents (Cl(OR)) in single acinar cells isolated from the rabbit superior lacrimal gland (RSLG) to investigate the hypothesis that Cl(OR) may have a role in regulating the volume of RSLG acini...
- Anoctamin/TMEM16 family members are Ca2+-activated Cl- channelsH Criss Hartzell
Department of Cell Biology, Emory University School of Medicine, 615 Michael Street, 535 Whitehead Bldg, Atlanta, GA 30322, USA
J Physiol 587:2127-39. 2009..Some members of this family are up-regulated in a number of tumours and functional deficiency in others is linked to developmental defects...
- Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophyAmi Mankodi
Department of Neurology, School of Medicine and Dentistry, University of Rochester, Box 673, 601 Elmwood Avenue, New York 14642, USA
Mol Cell 10:35-44. 2002..We propose that a transdominant effect of mutant RNA on RNA processing leads to chloride channelopathy and membrane hyperexcitability in DM...
- GLC-3: a novel fipronil and BIDN-sensitive, but picrotoxinin-insensitive, L-glutamate-gated chloride channel subunit from Caenorhabditis elegansL Horoszok
Department of Biology and Biochemistry, University of Bath, Claverton Down, Bath BA2 7AY
Br J Pharmacol 132:1247-54. 2001..Expression of glc-3 cRNA in XENOPUS oocytes resulted in the formation of homo-oligomeric L-glutamate-gated chloride channels with robust and rapidly desensitizing currents, an EC(50) of 1.9+/-0.03 mM and a Hill coefficient of 1.5+/-0...
- Bestrophin gene mutations in patients with Best vitelliform macular dystrophyG M Caldwell
Department of Cancer Genetics, Roswell Park Cancer Institute, 666 Elm Street, Buffalo, New York 14263, USA
Genomics 58:98-101. 1999..Finally, we have evidence that some mutations are associated with variable expression of the disease, suggesting the involvement of other factors or genes in the disease phenotype...
- Genetic CLC-1 chloride channel deficiency modifies diaphragm muscle isometric contractile propertiesErik van Lunteren
Pulmonary and Critical Care Medicine, Case Western Reserve University, Louis Stokes Cleveland Department of Veterans Affairs, Cleveland, OH 44106, USA
Respir Physiol Neurobiol 155:220-6. 2007..These findings indicate that genetic CLC-1 deficiency not only affects muscle relaxation (myotonia) but also modulates diaphragm performance during the contractile phase of the contraction-relaxation cycle...
- The avermectin receptors of Haemonchus contortus and Caenorhabditis elegansDarran M Yates
Department of Biology and Biochemistry, University of Bath, Claverton Down, BA2 7AY, Bath, UK
Int J Parasitol 33:1183-93. 2003..that the avermectin/milbemycin family of anthelmintics act via specific interactions with glutamate-gated chloride channels. These channels are encoded by a small family of genes in nematodes, though the composition of the gene ..
- Emerging roles of chloride channels in human diseasesLivia Puljak
Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390-8887, USA
Biochim Biophys Acta 1762:404-13. 2006..This review discusses the proposed roles of abnormal Cl(-) channel activity in the pathogenesis of human diseases...
- Morphological and functional changes in multifocal vitelliform retinopathy and biallelic mutations in BEST1Elisabeth Wittström
Department of Ophthalmology, Lund University, Sweden
Ophthalmic Genet 32:83-96. 2011..To describe morphological and functional changes in a single patient with multifocal Best vitelliform macular dystrophy (BVMD) and to perform a genotype/phenotype correlation...
- Functional roles of bestrophins in ocular epitheliaAlan D Marmorstein
Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ 85711, USA
Prog Retin Eye Res 28:206-26. 2009....
- Enhanced accumulation of A2E in individuals homozygous or heterozygous for mutations in BEST1 (VMD2)B Bakall
Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ 85724, USA
Exp Eye Res 85:34-43. 2007..Finally, the changes we observe in the granules suggest that the histopathology and eventual vision loss associated with BMD may be due to defects in the ability of the RPE to fully degrade phagocytosed photoreceptor outer segments...
- TMEM16B, a novel protein with calcium-dependent chloride channel activity, associates with a presynaptic protein complex in photoreceptor terminalsHeidi Stohr
Institut fur Humangenetik, Universitat Regensburg, 93053 Regensburg, Germany
J Neurosci 29:6809-18. 2009..The compartmentalized localization and the electrophysiological properties suggest TMEM16B to be a strong candidate for the long sought-after Ca(2+)-dependent chloride channel in the photoreceptor synapse...
- Chloride conductance in the transverse tubular system of rat skeletal muscle fibres: importance in excitation-contraction coupling and fatigueT L Dutka
Department of Zoology, La Trobe University, Melbourne, Victoria, Australia
J Physiol 586:875-87. 2008..When the t-system chloride conductance was eliminated, either by removal of all Cl(-) or by block of the chloride channels with 9-anthracene carboxylic acid (9-AC) or by treating muscles with phorbol 12,13-dibutyrate, there was a ..
- Differential macular and peripheral expression of bestrophin in human eyes and its implication for best diseaseRobert F Mullins
Carver Family Center for Macular Degeneration, Department of Ophthalmology and Visual Science, The University of Iowa Carver College of Medicine, Iowa City, Iowa 52242, USA
Invest Ophthalmol Vis Sci 48:3372-80. 2007..The nature of the vitelliform material and the reason the development of such lesions is usually restricted to the macula are two unsolved questions in the pathogenesis of this disorder...
- Chloride channels as drug targetsAlan S Verkman
Departments of Medicine and Physiology, University of California, San Francisco, California 94143 0521, USA
Nat Rev Drug Discov 8:153-71. 2009b>Chloride channels represent a relatively under-explored target class for drug discovery as elucidation of their identity and physiological roles has lagged behind that of many other drug targets...
- The Drosophila tweety family: molecular candidates for large-conductance Ca2+-activated Cl- channelsMakoto Suzuki
Department of Pharmacology, Division of Molecular Pharmacology, Jichi Medical School 3311 1, Yakushiji, Minamikawachi, Tochigi, 329 0498, Japan
Exp Physiol 91:141-7. 2006..I conclude that investigation of the tweety family will provide important information about large-conductance Cl- channel molecules...
- Bestrophins and retinopathiesQinghuan Xiao
Department of Cell Biology and Center for Neurodegenerative Disease, Emory University School of Medicine, Atlanta, GA 30322, USA
Pflugers Arch 460:559-69. 2010....
- Intracellular acidosis enhances the excitability of working muscleThomas H Pedersen
Department of Physiology, University of Aarhus, DK-8000, Denmark
Science 305:1144-7. 2004..These results implicate chloride ion channels in muscle function and emphasize that intracellular acidosis of muscle has protective effects during muscle fatigue...
- A muscleblind knockout model for myotonic dystrophyRahul N Kanadia
Department of Molecular Genetics and Microbiology, Powell Gene Therapy Center, Gainesville, FL 32610, USA
Science 302:1978-80. 2003..Our results support the hypothesis that manifestations of DM can result from sequestration of specific RNA binding proteins by a repetitive element expansion in a mutant RNA...
- Expression and function of epithelial anoctaminsRainer Schreiber
Institut fur Physiologie, Universitat Regensburg, Universitatsstrasse 31, D 93053 Regensburg, Germany
J Biol Chem 285:7838-45. 2010..We conclude that each tissue expresses a set of anoctamins that form cell- and tissue-specific Ca(2+)-dependent Cl(-) channels...
- Presence of a calcium-activated chloride current in mouse ventricular myocytesYanfang Xu
Division of Cardiovascular Medicine, Department of Medicine, University of California, Davis, California 95616, USA
Am J Physiol Heart Circ Physiol 283:H302-14. 2002..Niflumic acid (in the presence of 4-AP) leads to prolongation of the early repolarization. Thus this current may contribute to early repolarization of action potentials in mouse ventricular myocytes...
- Activation of the basolateral membrane Cl- conductance essential for electrogenic K+ secretion suppresses electrogenic Cl- secretionQuanhua He
Department of Neuroscience, Cell Biology and Physiology, Wright State University Boonshoft School of Medicine, 3640 Colonel Glenn Highway, Dayton, OH 45435, USA
Exp Physiol 96:305-16. 2011..Maximal physiological Cl(-) secretion occurs only for synergistic activation mechanisms that close these basolateral membrane Cl(-) channels...
- Detection of ClC-3 and ClC-5 in epididymal epithelium: immunofluorescence and RT-PCR after LCMCorinne Isnard-Bagnis
Renal Unit and Program in Membrane Biology, Massachusetts General Hospital, 149 13th Street, Charlestown, MA 02129, USA
Am J Physiol Cell Physiol 284:C220-32. 2003..ClC-5 might facilitate endosome trafficking in the epididymis, as has been proposed in the kidney...
- Dual effect of blocking agents on Ca2+-activated Cl(-) currents in rabbit pulmonary artery smooth muscle cellsA S Piper
Department of Pharmacology and Clinical Pharmacology, Cardiovascular Research Group, St George s Hospital Medical School, Cranmer Terrace, London SW17 0RE, UK
J Physiol 539:119-31. 2002..These results suggest that NFA and DCDPC, but not DIDS, simultaneously enhance and block I(Cl(Ca)) by binding to an external site, probably close to the mouth of the chloride channel...
- Contribution of chloride channels to volume regulation of cortical astrocytesKimberly A Parkerson
Department of Neurobiology, Civitan International Research Center, University of Alabama at Birmingham, Birmingham, Alabama 35294, USA
Am J Physiol Cell Physiol 284:C1460-7. 2003..These data suggest that Cl(-) flux through ion channels rather than transporters is essential for volume regulation of cultured astrocytes in response to hypotonic challenge...
- hCLCA1 and mCLCA3 are secreted non-integral membrane proteins and therefore are not ion channelsAdele Gibson
GlaxoSmithKline, Gunnels Wood Road, Stevenage, Hertfordshire SG1 2NY, United Kingdom
J Biol Chem 280:27205-12. 2005..We conclude that hCLCA1 and mCLCA3 are non-integral membrane proteins and therefore cannot be chloride channels in their own right.
- Myotonia-related mutations in the distal C-terminus of ClC-1 and ClC-0 chloride channels affect the structure of a poly-proline helixMaria J Macias
Institut de Recerca Biomèdica, Parc Cientific de Barcelona, Josep Samitier 1 5, Barcelona, E 08028, Spain
Biochem J 403:79-87. 2007..We provide the first experimental investigation of structural changes resulting from myotonia-causing mutations...
- Structure and function of CLCA proteinsMatthew E Loewen
Veterinary Biomedical Sciences, University of Saskatchewan, Saskatoon, Canada
Physiol Rev 85:1061-92. 2005..expression system rather than the CLCA protein suggests that these proteins are not independent Ca2+-dependent chloride channels, but may contribute to the activity of chloride channels formed by, or in conjunction with, other proteins.
- Relative contribution of chloride channels and transporters to regulatory volume decrease in human glioma cellsNola Jean Ernest
Department of Neurobiology, University of Alabama at Birmingham, USA
Am J Physiol Cell Physiol 288:C1451-60. 2005..Finally, we identified several candidate proteins that may be involved in RVD, including the Cl(-) channels ClC-2, ClC-3, ClC-5, ClC-6, and ClC-7 and the transporters KCC1 and KCC3a...
- Agonist enhacement of macrocyclic lactone activity at a glutamate-gated chloride channel subunit from Haemonchus contortusSean G Forrester
Institute of Parasitology, McGill University, 21, 111 Lakeshore Rd, Ste Anne de Bellevue, Que, Canada H9X 3V9
Biochem Pharmacol 67:1019-24. 2004..048 ). These results demonstrate that a GluCl agonist enhances IVM activity and provides further information on the mode of action of ivermectin in parasitic nematodes...
- Permeant anions control gating of calcium-dependent chloride channelsP Perez-Cornejo
School of Medicine, Universidad Autonoma de San Luis Potosi, 78290, Mexico
J Membr Biol 198:125-33. 2004..Thus, interactions of permeant anions with these two sites hinder the closing mechanism and stabilize the channel in the open state...
- The tyrosine kinase p56lck mediates activation of swelling-induced chloride channels in lymphocytesA Lepple-Wienhues
Department of Physiology, University of Tubingen, D 72076 Tubingen, Germany
J Cell Biol 141:281-6. 1998..In accordance with our patch-clamp data, osmotic swelling increases activity of immunoprecipitated p56lck. We conclude that osmotic swelling activates ICl-swell in lymphocytes via the tyrosine kinase p56lck...
- Mechanisms of Disease: the kidney-specific chloride channels ClCKA and ClCKB, the Barttin subunit, and their clinical relevanceBernhard K Krämer
Nephrology Renal Transplantation, Klinik und Poliklinik für Innere Medizin II Nephrologie at the University of Regensburg, Regensburg, Germany
Nat Clin Pract Nephrol 4:38-46. 2008Rodent ClC-K1 and ClC-K2, and their respective human orthologs ClCKA and ClCKB, are chloride channels specific to the kidney (and inner ear); Barttin is their functionally important subunit...
- Effect of bestrophin-1 on L-type Ca2+ channel activity depends on the Ca2+ channel beta-subunitNadine Reichhart
Experimental Ophthalmology, Eye Hospital, University Medical Center Regensburg, Franz Josef Straus Allee 11, 93053 Regensburg, Germany
Exp Eye Res 91:630-9. 2010..A disturbed interaction reduces the currents of the Ca(V)1.3 subunits. This mechanism could open new ways to understand changes in the patient's electro-oculogram and functional alterations of the RPE leading to retinal degeneration...
- A single beta subunit M2 domain residue controls the picrotoxin sensitivity of alphabeta heteromeric glycine receptor chloride channelsQ Shan
Department of Physiology and Pharmacology, University of Queensland, Brisbane, Queensland, Australia
J Neurochem 76:1109-20. 2001..This provides further evidence for an allosteric mechanism of picrotoxin inhibition, but renders it unlikely that picrotoxin (as an allosterically acting 'competitive' antagonist) binds to this residue...
- Transmembrane protein 16A (TMEM16A) is a Ca2+-regulated Cl- secretory channel in mouse airwaysJason R Rock
Department of Cell Biology, Duke University Medical Center, Durham, North Carolina 27708, USA
J Biol Chem 284:14875-80. 2009..Our data suggest that TMEM16A CaCC-mediated Cl(-) secretion appears to be necessary for normal airway surface liquid homeostasis...
- The TMEM16 protein family: a new class of chloride channels?Luis J V Galietta
Laboratory of Molecular Genetics, Istituto Giannina Gaslini, Genoa, Italy
Biophys J 97:3047-53. 2009..The roles of the other TMEM16 proteins are still unknown. The study of TMEM16 proteins may lead to identification of novel molecular mechanisms underlying ion transport and channel gating by voltage and Ca(2+)...
- Role of cardiac chloride currents in changes in action potential characteristics and arrhythmiasM Hiraoka
Department of Cardiovascular Diseases, Tokyo Medical and Dental University, Japan
Cardiovasc Res 40:23-33. 1998..Therefore, the contribution of Cl- currents to the genesis of arrhythmias may depend on their association with the conductance of other ions, especially that of K+...
- Intrarenal and subcellular localization of rat CLC5V A Luyckx
Renal Division, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA
Am J Physiol 275:F761-9. 1998..These findings are consistent with a model in which CLC5 encodes an endosomal chloride channel that facilitates acidification and trafficking of renal epithelial endosomes...
- Ligand-gated chloride channels are receptors for biogenic amines in C. elegansNiels Ringstad
Howard Hughes Medical Institute, Department of Biology, and McGovern Institute for Brain Research, MIT, Cambridge, MA 02139, USA
Science 325:96-100. 2009..We have identified in the nematode Caenorhabditis elegans three ligand-gated chloride channels that are receptors for biogenic amines: LGC-53 is a high-affinity dopamine receptor, LGC-55 is a high-..
- TMEM16B induces chloride currents activated by calcium in mammalian cellsSimone Pifferi
International School for Advanced Studies, Scuola Internazionale Superiore di Studi Avanzati SISSA, Via Beirut 2, 34014, Trieste, Italy
Pflugers Arch 458:1023-38. 2009..Our results demonstrate that mTMEM16B functions as a Ca(2+)-activated Cl(-) channel when expressed in HEK 293T cells...
- A voltage-dependent Ca2+ influx pathway regulates the Ca2+-dependent Cl(-) conductance of renal IMCD-3 cellsJohn E Linley
Epithelial Research Group, Institute for Cell and Molecular Biosciences, Medical School, Newcastle University, Newcastle upon Tyne NE2 4HH, UK
J Membr Biol 230:57-68. 2009..We speculate that during sustained membrane depolarization, calcium influx activates ICLCA which functions to modulate NaCl transport across the apical membrane of IMCD cells...
- Ion permeation and selectivity in ClC-type chloride channelsC Fahlke
Institute of Physiology, RWTH Aachen, Pauwelsstr 30, 52057 Aachen, Germany
Am J Physiol Renal Physiol 280:F748-57. 2001..Recently, a novel gene family encoding voltage-gated chloride channels, the ClC family, was identified...
- Alpha-AP-2 directs myosin VI-dependent endocytosis of cystic fibrosis transmembrane conductance regulator chloride channels in the intestineAnne Collaco
Department of Pediatrics and Cell Biology University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15206, USA
J Biol Chem 285:17177-87. 2010..The findings support alpha-AP-2 in directing myosin VI-dependent endocytosis of CFTR and a requirement for myosin VI in membrane invagination and coated pit formation in enterocytes...
- Cloning and expression of a protein kinase C-regulated chloride channel abundantly expressed in rat brain neuronal cellsM Kawasaki
Second Department of Internal Medicine, Tokyo Medical and Dental University, School of Medicine, Japan
Neuron 12:597-604. 1994..frame encodes 760 amino acids, which possess significantly amino acid identity with previously cloned CIC chloride channels. The chloride currents expressed in Xenopus oocytes injected with CIC-3 cRNA were completely blocked by ..
- Phenotypic variability in a French family with a novel mutation in the BEST1 gene causing multifocal best vitelliform macular dystrophyEmmanuelle Lacassagne
Universite Paris Descartes, Faculté de Médecine Paris Descartes site Necker, CERTO, Paris, France
Mol Vis 17:309-22. 2011..To describe genetic and clinical findings in a French family affected by best vitelliform macular dystrophy (BVMD)...
- Correction of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophyThurman M Wheeler
Department of Neurology, University of Rochester, Rochester, New York 14618, USA
J Clin Invest 117:3952-7. 2007....
- Calcium concentration jumps reveal dynamic ion selectivity of calcium-activated chloride currents in mouse olfactory sensory neurons and TMEM16b-transfected HEK 293T cellsClaudia Sagheddu
International School for Advanced Studies, Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy
J Physiol 588:4189-204. 2010....
- Mechanism of the excitatory Cl- response in mouse olfactory receptor neuronsJohannes Reisert
Department of Neuroscience, Johns Hopkins School of Medicine, 725 North Wolfe Street, Baltimore, Maryland 21205, USA
Neuron 45:553-61. 2005..Thus, a single functional architecture serves two entirely different purposes, probably underscoring the epithelial origin of the ORNs...
- The structural basis of ClC chloride channel functionRaimund Dutzler
Department of Biochemistry, University of Zurich, Winterthurerstrasse 190, CH 8057 Zurich, Switzerland
Trends Neurosci 27:315-20. 2004..In the closed conformation of the channel, a conserved glutamate residue occupies one of the ion-binding sites; on channel opening, this glutamate residue moves out of the binding site, thereby making room for an additional Cl(-) ion...
- Modulation of aqueous humor outflow by ionic mechanisms involved in trabecular meshwork cell volume regulationDavid Soto
Laboratory of Neurophysiology, Department of Physiological Sciences I-Institute of Biomedical Investigations August Pi i Sunyer, University of Barcelona, Spain
Invest Ophthalmol Vis Sci 45:3650-61. 2004....
- Inhibition of swelling-activated Cl- currents by functional anti-ClC-3 antibody in native bovine non-pigmented ciliary epithelial cellsChi Wai Do
Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
Invest Ophthalmol Vis Sci 46:948-55. 2005..This information will be useful in understanding the mechanisms controlling aqueous humor formation and thereby intraocular pressure...
- The shift of the reversal potential of glutamate-activated chloride current in molluscan neurons caused by 8-Br-cAMPE I Solntseva
Brain Research Institute, Russian Academy of Medical Sciences, per. Obukha 5, Moscow, 105064, Russia
Dokl Biol Sci 399:447-50. 2004
- Bradykinin decreases K(+) and increases Cl(-) conductances in vagal afferent neurones of the guinea pigEun Joo Oh
University of Maryland School of Medicine, Department of Pharmacology and Experimental Therapeutics, Room 4-002, Bressler Research Building, 655 West Baltimore Street, Baltimore, MD 21201-1559, USA
J Physiol 558:513-26. 2004..The BK-evoked Cl(-) conductance in NGNs may be the first demonstration of an inflammatory mediator exciting primary afferents via an anion channel...
- SPI-0211 activates T84 cell chloride transport and recombinant human ClC-2 chloride currentsJohn Cuppoletti
Department of Molecular and Cellular Physiology, College of Medicine, University of Cincinnati, Cincinnati, Ohio 45267 0576, USA
Am J Physiol Cell Physiol 287:C1173-83. 2004..Together, these studies demonstrate that SPI-0211 is a potent activator of ClC-2 Cl- channels and suggest a physiologically relevant role for ClC-2 Cl- channels in intestinal Cl- transport after SPI-0211 administration...
- Effect of the flavonol quercetin on membrane conductances in rat colonic crypt cellsRainer Cermak
Institute of Animal Nutrition and Physiology, Christian Albrechts University of Kiel, Olshausenstr 40, D 24098 Kiel, Germany
Eur J Pharmacol 503:31-4. 2004..Thus, quercetin induces Cl- secretion merely by activation of K+ channels in the colon epithelium...
- Chloride equilibrium potential in salamander conesWallace B Thoreson
Department of Ophthalmology, University of Nebraska Medical Center, Omaha, NE, USA
BMC Neurosci 5:53. 2004..To assess the impact of these and other chloride-dependent mechanisms on release from cones, the chloride equilibrium potential (ECl) was determined in red-sensitive, large single cones from the tiger salamander retinal slice...
- Electrophysiological characterization of volume-activated chloride currents in mouse cholangiocyte cell lineBiyi Chen
Department of Medicine, Division of Gatroenterology/Hepatology, Indiana University School of Medicine, Indianapolis, IN 46202, USA
Am J Physiol Gastrointest Liver Physiol 287:G1158-67. 2004..The present study is the first to characterize the VACC in mouse cholangiocyte and will provide an important framework for further studies to examine and understand the role of the VACC in biliary secretion and ion-transport physiology...
- Activation of chloride currents in murine portal vein smooth muscle cells by membrane depolarization involves intracellular calcium releaseSohag N Saleh
Department of Basic Medical Sciences, Pharmacology and Clinical Pharmacology, St. George's Hospital Medical School, London, United Kingdom
Am J Physiol Cell Physiol 288:C122-31. 2005..In isometric tension recordings, NFA inhibited spontaneous contractions. These data support a role for this conductance in portal vein excitability...
- Chloride channels in the small intestinal cell line IEC-18Srisaila Basavappa
Digestive Diseases Unit, Department of Medicine, University of Rochester School of Medicine Rochester, New York 14642, USA
J Cell Physiol 202:21-31. 2005..These results suggest that small intestinal crypt cells express multiple types of Cl- channels, which may all contribute to net Cl- secretion...
- Chloride channel blockers activate an endogenous cationic current in oocytes of Bufo arenarumM S Cavarra
Facultad de Medicina (UBA, , C. de Malvinas 3150, 1427 Buenos Aires, Argentina
J Comp Physiol A Neuroethol Sens Neural Behav Physiol 190:531-7. 2004....
- Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteinsOlaf Scheel
, ZMNH, , Falkenried 94, D-20246 Hamburg, Germany
Nature 436:424-7. 2005Eukaryotic members of the CLC gene family function as plasma membrane chloride channels, or may provide neutralizing anion currents for V-type H(+)-ATPases that acidify compartments of the endosomal/lysosomal pathway...
- Small-molecule screen identifies inhibitors of a human intestinal calcium-activated chloride channelRicardo de la Fuente
1246 Health Sciences East Tower, Box 0521, University of California, San Francisco CA 94143 0521, USA
Mol Pharmacol 73:758-68. 2008Calcium-activated chloride channels (CaCCs) are widely expressed in mammalian tissues, including intestinal epithelia, where they facilitate fluid secretion. Potent, selective CaCC inhibitors have not been available...
- Effects of increased intracellular Cl- concentration on membrane responses to acetylcholine in the isolated endothelium of guinea pig mesenteric arteriesYoshimichi Yamamoto
Laboratory of Physiology, Nagoya City University School of Nursing, Mizuho ku, Nagoya, 467 8601, Japan
J Physiol Sci 57:31-41. 2007..Such a variability in [Cl(-)](i) may be one of the reasons for the variations in the ACh-induced membrane response...
- Regulation of the subthreshold chloride conductance in the rat sympathetic neuronOscar Sacchi
Department of Biology, Section of Physiology and Biophysics and Center of Neuroscience, Ferrara University, Via Borsari, 46, I 44100 Ferrara, Italy
Eur J Neurosci 25:1112-26. 2007..Sensitization was transient in response to neuron activity. In the silent neuron, sensitization of gCl shifted membrane potential over a range of about 15 mV...
- Temporal development of cyclic nucleotide-gated and Ca2+ -activated Cl- currents in isolated mouse olfactory sensory neuronsAnna Boccaccio
International School for Advanced Studies, SISSA, Sector of Neurobiology, Basovizza, Trieste, Italy
J Neurophysiol 98:153-60. 2007..These data show how to unravel the CNG and Ca(2+)-activated Cl(-) component of the current rising phase...
- ClC-3 is required for LPA-activated Cl- current activity and fibroblast-to-myofibroblast differentiationZhaohong Yin
Department of Physiology, University of Tennessee Health Science Center, 894 Union Ave, Memphis, TN 38163, USA
Am J Physiol Cell Physiol 294:C535-42. 2008..These data confirm that ClC-3 is important in VRAC function and cell volume regulation, is associated with the I(Cl,LPA) current activity, and participates in the fibroblast-to-myofibroblast transition...
- Regulation of excitation by glycine receptorsJiang Hong Ye
Department of Anesthesiology, UMDNJ, New Jersey Medical School, Newark, NJ 07103, USA
Results Probl Cell Differ 44:123-43. 2008..This chapter is an overview of selective studies on the newly discovered roles of GlyRs in regulating neuronal excitation, and inhibition, particularly in the upper brain areas...
- The mechanism of fast-gate opening in ClC-0Anita M Engh
Department of Molecular and Cellular Physiology, Stanford University School of Medicine, Stanford, CA 94305, USA
J Gen Physiol 130:335-49. 2007....
- Functional ion channels in mouse bone marrow mesenchymal stem cellsRong Tao
Department of Medicine and Research Center of Heart, Brain, Hormone and Healthy Aging, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Pokfulam, Hong Kong SAR, China
Am J Physiol Cell Physiol 293:C1561-7. 2007..These results demonstrate that three types of functional ion channel currents (i.e., I(Kir), I(KCa), and I(Cl)) are present in mouse bone marrow MSCs...
- ClC3 is a critical regulator of the cell cycle in normal and malignant glial cellsChrista W Habela
Department of Neurobiology, Center for Glial Biology in Medicine, University of Alabama at Birmingham, Birmingham, Alabama 35294, USA
J Neurosci 28:9205-17. 2008..These data suggest that PMC is an integral part of cell division and is dependent on ClC3 channel function...
- Extracellular zinc stimulates a calcium-activated chloride conductance through mobilisation of intracellular calcium in renal inner medullary collecting duct cellsJ E Linley
Institute of Cell and Molecular Biosciences, University Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK
Pflugers Arch 453:487-95. 2007..A possible physiological role for a divalent metal ion receptor, distinct from the extracellular Ca(2+)-sensing receptor, in IMCD cells is discussed...
- Flow-activated chloride channels in vascular endothelium. Shear stress sensitivity, desensitization dynamics, and physiological implicationsMamta Gautam
Department of Mechanical and Aeronautical Engineering, University of California, Davis, California 95616, USA
J Biol Chem 281:36492-500. 2006....
- Association between Hsp90 and the ClC-2 chloride channel upregulates channel functionAlexandre Hinzpeter
INSERM, Unité 467, Faculte de Medecine Necker Enfants Malades, 156 rue de Vaugirard, 75015 Paris, France
Am J Physiol Cell Physiol 290:C45-56. 2006..This association may have important pathophysiological consequences, enabling increased ClC-2 activity in response to cellular stresses such as elevated temperature, ischemia, or oxidative reagents...
- Volume-activated chloride currents in interstitial cells of CajalSung Jin Park
McMaster University, Intestinal Disease Programme, Department of Medicine, Hamilton, Ontario, Canada
Am J Physiol Gastrointest Liver Physiol 289:G791-7. 2005..In conclusion, swelling or volume change will depolarize ICC through activation of outwardly rectifying chloride channels, thereby increasing cell excitability.
- Altered gating and regulation of a carboxy-terminal ClC channel mutant expressed in the Caenorhabditis elegans oocyteJerod Denton
Department of Anesthesiology, Vanderbilt University Medical Center, T 4202 Medical Center North, Nashville, TN 37232 2520, USA
Am J Physiol Cell Physiol 290:C1109-18. 2006..Our findings contribute to a growing body of evidence indicating that cytoplasmic domains play key roles in the gating and regulation of eukaryotic ClCs...
- Glycine Channels and Hippocampal ExcitabilityLori McMahon; Fiscal Year: 2006An inhibitory role of strychnine-sensitive glycine-gated chloride channels (GlyRs) in the brain has largely been ignored, despite the known expression of these receptors throughout the CNS...
- Cholesterol and Sphingolipid Perturbation of Membrane Traffic in Human DiseaseRichard Pagano; Fiscal Year: 2009..The following studies will examine the role of these lipids in exacerbating the symptoms of Cystic fibrosis and will be used in developing new therapeutic approaches for this disease. ..
- Structure and function of chloride channels and transportersAlessio Accardi; Fiscal Year: 2009b>Chloride channels and transporters of the CLC family play crucial roles in a myriad of physiological processes including regulation of electrical excitability of nerve and muscle cells, modulation of salt and water movement across ..
- Structure and function of chloride channels and transportersAlessio Accardi; Fiscal Year: 2010b>Chloride channels and transporters of the CLC family play crucial roles in a myriad of physiological processes including regulation of electrical excitability of nerve and muscle cells, modulation of salt and water movement across ..
- GABAERGIC INSECTICIDE TOXICOLOGYJOHN CASIDA; Fiscal Year: 2002....
- Radiovirotherapy for Multiple MyelomaStephen J Russell; Fiscal Year: 2010..The studies to be pursued in this grant combine the promising novel approach of oncolytic virotherapy with the use of radioactive iodine for the treatment of this disease. ..
- Daily genistein injections stimulate intestinal secretion: How?Layla Al Nakkash; Fiscal Year: 2007..Furthermore, we aim to identify the suitability of genistein as a potential therapeutic for the most common lethal genetic disease in Caucasians, cystic fibrosis (CF). ..
- MECHANISMS OF CEREBRAL RESISTANCE ARTERY CONTRACTIONJoseph Brayden; Fiscal Year: 2000..Specific Aim #3 will test the hypothesis that chloride channels contribute to regulation of membrane potential in cerebral arteries...
- TGF-BETA, CHLORIDE CHANNELS AND MIGRATION OF EOSINOPHILSDevendra K Agrawal; Fiscal Year: 2010..The information obtained from this study should provide an opportunity to formulate superior therapeutic approaches in chronic asthma. ..
- Role of Myosin VI in CFTR Endocytosis in the IntestineNadia Ameen; Fiscal Year: 2007..anion secretion in the intestine. The additional funds provided by this award will enable the PI to expand investigations and develop collaborations as she pursues these revised specific aims. ..
- Cell Biology of CFTR in Polarized EpitheliaJames Collawn; Fiscal Year: 2009..In the proposed studies, we are examining the cell surface stability of this chloride channel and the cellular mechanisms that regulate its cell surface stability. ..
- Cell Biology of CFTR in Polarized EpitheliaJames F Collawn; Fiscal Year: 2010..In the proposed studies, we are examining the cell surface stability of this chloride channel and the cellular mechanisms that regulate its cell surface stability. ..
- CFTR Biogenesis and Function in EpitheliaZsuzsanna Bebok; Fiscal Year: 2009..Understanding these processes are important in understanding the cellular basis for a number of lung pathologies including cystic fibrosis and chronic obstructive pulmonary disease (COPD). ..
- CFTR Biogenesis and Function in EpitheliaZsuzsa Bebok; Fiscal Year: 2010..Understanding these processes are important in understanding the cellular basis for a number of lung pathologies including cystic fibrosis and chronic obstructive pulmonary disease (COPD). ..
- Tissue-specific regulation of a gene essential for normal airway epitheliaAnn Harris; Fiscal Year: 2010..Translational opportunities that may arise from this work include modulating CFTR expression in vivo and the construction of efficient, tissue-specific gene therapy vectors. ..
- Tissue-specific regulation of a gene essential for normal airway epitheliaAnn Harris; Fiscal Year: 2009..Translational opportunities that may arise from this work include modulating CFTR expression in vivo and the construction of efficient, tissue-specific gene therapy vectors. ..
- Mi: Modulating Osteoclast Gene Expression and FunctionMichael C Ostrowski; Fiscal Year: 2010..The genetics and molecular biology of MITF suggests that the MITF pathway may provide molecular targets for certain bone disorders. ..
- Mi: Modulating Osteoclast Gene Expression and FunctionMichael Ostrowski; Fiscal Year: 2009..The genetics and molecular biology of MITF suggests that the MITF pathway may provide molecular targets for certain bone disorders. ..
- Molecular Physiology of CLC Chloride ChannelsTsung Yu Chen; Fiscal Year: 2010..PUBLIC HEALTH RELEVANCE: This application will study CLC channels, which are chloride channels critical for chloride ion transports across cell membranes...
- Molecular Physiology of CLC Chloride ChannelsTsung Yu Chen; Fiscal Year: 2010..PUBLIC HEALTH RELEVANCE: This application will study CLC channels, which are chloride channels critical for chloride ion transports across cell membranes...
- Rescue of CFTR-deltaF508 misfolding by pharmacological and genetic suppressorsRYAN TYLER; Fiscal Year: 2007..The broad objective of our studies is to uncover leads that could translate into new treatments for cystic fibrosis. ..
- Physiologic membrane traffic regulation: intestinal CFTRNadia Ameen; Fiscal Year: 2006..intestinal tissues to determine a role for membrane traffic in regulated CFTR and other potential alternate chloride channels and anion transport...
- Limiting Inflammation in the CF LungPamela Davis; Fiscal Year: 2007..The name of the principal investigator/program director must be provided at the top of each printed page and each continuation page. RESEARCH GRANT TABLE OF CONTENTS PageNumbers Face Page ' Description, ..
- Cholesterol and Sphingolipid Perturbation of Membrane Traffic in Human DiseaseAndrew H Limper; Fiscal Year: 2011..The following studies will examine the role of these lipids in exacerbating the symptoms of Cystic fibrosis and will be used in developing new therapeutic approaches for this disease. ..
- Cholesterol and Sphingolipid Perturbation of Membrane Traffic in Human DiseaseAndrew H Limper; Fiscal Year: 2010..The following studies will examine the role of these lipids in exacerbating the symptoms of Cystic fibrosis and will be used in developing new therapeutic approaches for this disease. ..
- Functional Relevance of CFTR Trafficking in Vivo to Intestinal DiseaseNadia Ameen; Fiscal Year: 2009..This proposal seeks to understand the mechanisms that regulate CFTR movement into to the membrane under physiological conditions in the intestine in order to elucidate how dysfunction of CFTR leads to either CF or secretory diarrhea. ..
- Functional Relevance of CFTR Trafficking in Vivo to Intestinal DiseaseNadia A Ameen; Fiscal Year: 2010..This proposal seeks to understand the mechanisms that regulate CFTR movement into to the membrane under physiological conditions in the intestine in order to elucidate how dysfunction of CFTR leads to either CF or secretory diarrhea. ..
- CHLORIDE CHANNELS AND GASTRIC ACID SECRETIONJohn Cuppoletti; Fiscal Year: 1993..Preliminary results of inhibitor and pH sensitivity suggest that the recorded single Cl- channels may participate in HC1 accumulation...