Chloride Channels: Cell membrane glycoproteins that form channels to selectively pass chloride ions. Nonselective blockers include FENAMATES; ETHACRYNIC ACID; and TAMOXIFEN.Chlorides: Inorganic compounds derived from hydrochloric acid that contain the Cl- ion.Ion Channels: Gated, ion-selective glycoproteins that traverse membranes. The stimulus for ION CHANNEL GATING can be due to a variety of stimuli such as LIGANDS, a TRANSMEMBRANE POTENTIAL DIFFERENCE, mechanical deformation or through INTRACELLULAR SIGNALING PEPTIDES AND PROTEINS.Calcium Channels: Voltage-dependent cell membrane glycoproteins selectively permeable to calcium ions. They are categorized as L-, T-, N-, P-, Q-, and R-types based on the activation and inactivation kinetics, ion specificity, and sensitivity to drugs and toxins. The L- and T-types are present throughout the cardiovascular and central nervous systems and the N-, P-, Q-, & R-types are located in neuronal tissue.Ion Channel Gating: The opening and closing of ion channels due to a stimulus. The stimulus can be a change in membrane potential (voltage-gated), drugs or chemical transmitters (ligand-gated), or a mechanical deformation. Gating is thought to involve conformational changes of the ion channel which alters selective permeability.Cystic Fibrosis Transmembrane Conductance Regulator: A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)Potassium Channels, Inwardly Rectifying: Potassium channels where the flow of K+ ions into the cell is greater than the outward flow.Nitrobenzoates: Benzoic acid or benzoic acid esters substituted with one or more nitro groups.Calcium Channel Blockers: A class of drugs that act by selective inhibition of calcium influx through cellular membranes.Potassium Channel Blockers: A class of drugs that act by inhibition of potassium efflux through cell membranes. Blockade of potassium channels prolongs the duration of ACTION POTENTIALS. They are used as ANTI-ARRHYTHMIA AGENTS and VASODILATOR AGENTS.Myotonia Congenita: Inherited myotonic disorders with early childhood onset MYOTONIA. Muscular hypertrophy is common and myotonia may impair ambulation and other movements. It is classified as Thomsen (autosomal dominant) or Becker (autosomal recessive) generalized myotonia mainly based on the inheritance pattern. Becker type is also clinically more severe. An autosomal dominant variant with milder symptoms and later onset is known as myotonia levior. Mutations in the voltage-dependent skeletal muscle chloride channel are associated with the disorders.4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid: An inhibitor of anion conductance including band 3-mediated anion transport.Electrophysiology: The study of the generation and behavior of electrical charges in living organisms particularly the nervous system and the effects of electricity on living organisms.Membrane Potentials: The voltage differences across a membrane. For cellular membranes they are computed by subtracting the voltage measured outside the membrane from the voltage measured inside the membrane. They result from differences of inside versus outside concentration of potassium, sodium, chloride, and other ions across cells' or ORGANELLES membranes. For excitable cells, the resting membrane potentials range between -30 and -100 millivolts. Physical, chemical, or electrical stimuli can make a membrane potential more negative (hyperpolarization), or less negative (depolarization).Myotonia: Prolonged failure of muscle relaxation after contraction. This may occur after voluntary contractions, muscle percussion, or electrical stimulation of the muscle. Myotonia is a characteristic feature of MYOTONIC DISORDERS.Electric Conductivity: The ability of a substrate to allow the passage of ELECTRONS.Patch-Clamp Techniques: An electrophysiologic technique for studying cells, cell membranes, and occasionally isolated organelles. All patch-clamp methods rely on a very high-resistance seal between a micropipette and a membrane; the seal is usually attained by gentle suction. The four most common variants include on-cell patch, inside-out patch, outside-out patch, and whole-cell clamp. Patch-clamp methods are commonly used to voltage clamp, that is control the voltage across the membrane and measure current flow, but current-clamp methods, in which the current is controlled and the voltage is measured, are also used.Niflumic Acid: An analgesic and anti-inflammatory agent used in the treatment of rheumatoid arthritis.Potassium Channels, Voltage-Gated: Potassium channel whose permeability to ions is extremely sensitive to the transmembrane potential difference. The opening of these channels is induced by the membrane depolarization of the ACTION POTENTIAL.Calcium Channels, L-Type: Long-lasting voltage-gated CALCIUM CHANNELS found in both excitable and nonexcitable tissue. They are responsible for normal myocardial and vascular smooth muscle contractility. Five subunits (alpha-1, alpha-2, beta, gamma, and delta) make up the L-type channel. The alpha-1 subunit is the binding site for calcium-based antagonists. Dihydropyridine-based calcium antagonists are used as markers for these binding sites.Oocytes: Female germ cells derived from OOGONIA and termed OOCYTES when they enter MEIOSIS. The primary oocytes begin meiosis but are arrested at the diplotene state until OVULATION at PUBERTY to give rise to haploid secondary oocytes or ova (OVUM).KATP Channels: Heteromultimers of Kir6 channels (the pore portion) and sulfonylurea receptor (the regulatory portion) which affect function of the HEART; PANCREATIC BETA CELLS; and KIDNEY COLLECTING DUCTS. KATP channel blockers include GLIBENCLAMIDE and mitiglinide whereas openers include CROMAKALIM and minoxidil sulfate.Xenopus laevis: The commonest and widest ranging species of the clawed "frog" (Xenopus) in Africa. This species is used extensively in research. There is now a significant population in California derived from escaped laboratory animals.Anions: Negatively charged atoms, radicals or groups of atoms which travel to the anode or positive pole during electrolysis.Potassium Channels, Calcium-Activated: Potassium channels whose activation is dependent on intracellular calcium concentrations.Xenopus: An aquatic genus of the family, Pipidae, occurring in Africa and distinguished by having black horny claws on three inner hind toes.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Vinyl Chloride: A gas that has been used as an aerosol propellant and is the starting material for polyvinyl resins. Toxicity studies have shown various adverse effects, particularly the occurrence of liver neoplasms.Ion Transport: The movement of ions across energy-transducing cell membranes. Transport can be active, passive or facilitated. Ions may travel by themselves (uniport), or as a group of two or more ions in the same (symport) or opposite (antiport) directions.Shaker Superfamily of Potassium Channels: Voltage-gated potassium channels whose primary subunits contain six transmembrane segments and form tetramers to create a pore with a voltage sensor. They are related to their founding member, shaker protein, Drosophila.Sodium Channel Blockers: A class of drugs that act by inhibition of sodium influx through cell membranes. Blockade of sodium channels slows the rate and amplitude of initial rapid depolarization, reduces cell excitability, and reduces conduction velocity.Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.Sodium Chloride: A ubiquitous sodium salt that is commonly used to season food.Calcium Channels, N-Type: CALCIUM CHANNELS that are concentrated in neural tissue. Omega toxins inhibit the actions of these channels by altering their voltage dependence.Kinetics: The rate dynamics in chemical or physical systems.Ivermectin: A mixture of mostly avermectin H2B1a (RN 71827-03-7) with some avermectin H2B1b (RN 70209-81-3), which are macrolides from STREPTOMYCES avermitilis. It binds glutamate-gated chloride channel to cause increased permeability and hyperpolarization of nerve and muscle cells. It also interacts with other CHLORIDE CHANNELS. It is a broad spectrum antiparasitic that is active against microfilariae of ONCHOCERCA VOLVULUS but not the adult form.Large-Conductance Calcium-Activated Potassium Channels: A major class of calcium activated potassium channels whose members are voltage-dependent. MaxiK channels are activated by either membrane depolarization or an increase in intracellular Ca(2+). They are key regulators of calcium and electrical signaling in a variety of tissues.TRPC Cation Channels: A subgroup of TRP cation channels that contain 3-4 ANKYRIN REPEAT DOMAINS and a conserved C-terminal domain. Members are highly expressed in the CENTRAL NERVOUS SYSTEM. Selectivity for calcium over sodium ranges from 0.5 to 10.Calcium Channels, T-Type: A heterogenous group of transient or low voltage activated type CALCIUM CHANNELS. They are found in cardiac myocyte membranes, the sinoatrial node, Purkinje cells of the heart and the central nervous system.Hypotonic Solutions: Solutions that have a lesser osmotic pressure than a reference solution such as blood, plasma, or interstitial fluid.Cell Line: Established cell cultures that have the potential to propagate indefinitely.Epithelial Sodium Channels: Sodium channels found on salt-reabsorbing EPITHELIAL CELLS that line the distal NEPHRON; the distal COLON; SALIVARY DUCTS; SWEAT GLANDS; and the LUNG. They are AMILORIDE-sensitive and play a critical role in the control of sodium balance, BLOOD VOLUME, and BLOOD PRESSURE.Cell Membrane: The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.Potassium: An element in the alkali group of metals with an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte that plays a significant role in the regulation of fluid volume and maintenance of the WATER-ELECTROLYTE BALANCE.Cyclic Nucleotide-Gated Cation Channels: A subgroup of cyclic nucleotide-regulated ION CHANNELS within the superfamily of pore-loop cation channels. They are expressed in OLFACTORY NERVE cilia and in PHOTORECEPTOR CELLS and some PLANTS.Mutation: Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.Adenosine Triphosphate: An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.Cystic Fibrosis: An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.Acid Sensing Ion Channels: A family of proton-gated sodium channels that are primarily expressed in neuronal tissue. They are AMILORIDE-sensitive and are implicated in the signaling of a variety of neurological stimuli, most notably that of pain in response to acidic conditions.Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Kv1.3 Potassium Channel: A delayed rectifier subtype of shaker potassium channels that is the predominant VOLTAGE-GATED POTASSIUM CHANNEL of T-LYMPHOCYTES.Ether-A-Go-Go Potassium Channels: A family of voltage-gated potassium channels that are characterized by long N-terminal and C-terminal intracellular tails. They are named from the Drosophila protein whose mutation causes abnormal leg shaking under ether anesthesia. Their activation kinetics are dependent on extracellular MAGNESIUM and PROTON concentration.Kv1.2 Potassium Channel: A delayed rectifier subtype of shaker potassium channels that is selectively inhibited by a variety of SCORPION VENOMS.Glycolates: Derivatives of ACETIC ACID which contain an hydroxy group attached to the methyl carbon.Kv1.1 Potassium Channel: A delayed rectifier subtype of shaker potassium channels that is commonly mutated in human episodic ATAXIA and MYOKYMIA.Hydrogen-Ion Concentration: The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)Polyvinyl Chloride: A polyvinyl resin used extensively in the manufacture of plastics, including medical devices, tubing, and other packaging. It is also used as a rubber substitute.Membrane Proteins: Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.Glyburide: An antidiabetic sulfonylurea derivative with actions similar to those of chlorpropamide.Bartter Syndrome: A group of disorders caused by defective salt reabsorption in the ascending LOOP OF HENLE. It is characterized by severe salt-wasting, HYPOKALEMIA; HYPERCALCIURIA; metabolic ALKALOSIS, and hyper-reninemic HYPERALDOSTERONISM without HYPERTENSION. There are several subtypes including ones due to mutations in the renal specific SODIUM-POTASSIUM-CHLORIDE SYMPORTERS.TRPM Cation Channels: A subgroup of TRP cation channels named after melastatin protein. They have the TRP domain but lack ANKYRIN repeats. Enzyme domains in the C-terminus leads to them being called chanzymes.Calcium Channel Agonists: Agents that increase calcium influx into calcium channels of excitable tissues. This causes vasoconstriction in VASCULAR SMOOTH MUSCLE and/or CARDIAC MUSCLE cells as well as stimulation of insulin release from pancreatic islets. Therefore, tissue-selective calcium agonists have the potential to combat cardiac failure and endocrinological disorders. They have been used primarily in experimental studies in cell and tissue culture.Kv1.5 Potassium Channel: A delayed rectifier subtype of shaker potassium channels that conducts a delayed rectifier current. It contributes to ACTION POTENTIAL repolarization of MYOCYTES in HEART ATRIA.Sodium: A member of the alkali group of metals. It has the atomic symbol Na, atomic number 11, and atomic weight 23.TRPV Cation Channels: A subgroup of TRP cation channels named after vanilloid receptor. They are very sensitive to TEMPERATURE and hot spicy food and CAPSAICIN. They have the TRP domain and ANKYRIN repeats. Selectivity for CALCIUM over SODIUM ranges from 3 to 100 fold.Receptors, Glycine: Cell surface receptors that bind GLYCINE with high affinity and trigger intracellular changes which influence the behavior of cells. Glycine receptors in the CENTRAL NERVOUS SYSTEM have an intrinsic chloride channel and are usually inhibitory.Protein Structure, Tertiary: The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.Iodides: Inorganic binary compounds of iodine or the I- ion.Dose-Response Relationship, Drug: The relationship between the dose of an administered drug and the response of the organism to the drug.KCNQ Potassium Channels: A family of delayed rectifier voltage-gated potassium channels that share homology with their founding member, KCNQ1 PROTEIN. KCNQ potassium channels have been implicated in a variety of diseases including LONG QT SYNDROME; DEAFNESS; and EPILEPSY.Cell Size: The quantity of volume or surface area of CELLS.Shab Potassium Channels: A subfamily of shaker potassium channels that shares homology with its founding member, Shab protein, Drosophila. They regulate delayed rectifier currents in the NERVOUS SYSTEM of DROSOPHILA and in the SKELETAL MUSCLE and HEART of VERTEBRATES.Transfection: The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.KCNQ1 Potassium Channel: A voltage-gated potassium channel that is expressed primarily in the HEART.Rats, Sprague-Dawley: A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.Neurons: The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.Ions: An atom or group of atoms that have a positive or negative electric charge due to a gain (negative charge) or loss (positive charge) of one or more electrons. Atoms with a positive charge are known as CATIONS; those with a negative charge are ANIONS.Small-Conductance Calcium-Activated Potassium Channels: A major class of calcium-activated potassium channels that are found primarily in excitable CELLS. They play important roles in the transmission of ACTION POTENTIALS and generate a long-lasting hyperpolarization known as the slow afterhyperpolarization.Barium: An element of the alkaline earth group of metals. It has an atomic symbol Ba, atomic number 56, and atomic weight 138. All of its acid-soluble salts are poisonous.Scorpion Venoms: Venoms from animals of the order Scorpionida of the class Arachnida. They contain neuro- and hemotoxins, enzymes, and various other factors that may release acetylcholine and catecholamines from nerve endings. Of the several protein toxins that have been characterized, most are immunogenic.Cell Membrane Permeability: A quality of cell membranes which permits the passage of solvents and solutes into and out of cells.Kv1.4 Potassium Channel: A fast inactivating subtype of shaker potassium channels that contains two inactivation domains at its N terminus.Mercuric Chloride: Mercury chloride (HgCl2). A highly toxic compound that volatizes slightly at ordinary temperature and appreciably at 100 degrees C. It is corrosive to mucous membranes and used as a topical antiseptic and disinfectant.Intermediate-Conductance Calcium-Activated Potassium Channels: A major class of calcium-activated potassium channels that were originally discovered in ERYTHROCYTES. They are found primarily in non-excitable CELLS and set up electrical gradients for PASSIVE ION TRANSPORT.Transient Receptor Potential Channels: A broad group of eukaryotic six-transmembrane cation channels that are classified by sequence homology because their functional involvement with SENSATION is varied. They have only weak voltage sensitivity and ion selectivity. They are named after a DROSOPHILA mutant that displayed transient receptor potentials in response to light. A 25-amino-acid motif containing a TRP box (EWKFAR) just C-terminal to S6 is found in TRPC, TRPV and TRPM subgroups. ANKYRIN repeats are found in TRPC, TRPV & TRPN subgroups. Some are functionally associated with TYROSINE KINASE or TYPE C PHOSPHOLIPASES.Models, Biological: Theoretical representations that simulate the behavior or activity of biological processes or diseases. For disease models in living animals, DISEASE MODELS, ANIMAL is available. Biological models include the use of mathematical equations, computers, and other electronic equipment.ortho-Aminobenzoates: Benzoic acids, salts, or esters that contain an amino group attached to carbon number 2 or 6 of the benzene ring structure.Tetraethylammonium: A potassium-selective ion channel blocker. (From J Gen Phys 1994;104(1):173-90)Cricetinae: A subfamily in the family MURIDAE, comprising the hamsters. Four of the more common genera are Cricetus, CRICETULUS; MESOCRICETUS; and PHODOPUS.Mesylates: Organic salts or esters of methanesulfonic acid.Shaw Potassium Channels: A shaker subfamily that is prominently expressed in NEURONS and are necessary for high-frequency, repetitive firing of ACTION POTENTIALS.Receptors, GABA: Cell-surface proteins that bind GAMMA-AMINOBUTYRIC ACID with high affinity and trigger changes that influence the behavior of cells. GABA-A receptors control chloride channels formed by the receptor complex itself. They are blocked by bicuculline and usually have modulatory sites sensitive to benzodiazepines and barbiturates. GABA-B receptors act through G-proteins on several effector systems, are insensitive to bicuculline, and have a high affinity for L-baclofen.2-Methyl-4-chlorophenoxyacetic Acid: A powerful herbicide used as a selective weed killer.Mutagenesis, Site-Directed: Genetically engineered MUTAGENESIS at a specific site in the DNA molecule that introduces a base substitution, or an insertion or deletion.Binding Sites: The parts of a macromolecule that directly participate in its specific combination with another molecule.Action Potentials: Abrupt changes in the membrane potential that sweep along the CELL MEMBRANE of excitable cells in response to excitation stimuli.Cations: Positively charged atoms, radicals or groups of atoms which travel to the cathode or negative pole during electrolysis.Kidney: Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.Recombinant Proteins: Proteins prepared by recombinant DNA technology.Anthracenes: A group of compounds with three aromatic rings joined in linear arrangement.Models, Molecular: Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.Shal Potassium Channels: A shaker subfamily of potassium channels that participate in transient outward potassium currents by activating at subthreshold MEMBRANE POTENTIALS, inactivating rapidly, and recovering from inactivation quickly.Protein Subunits: Single chains of amino acids that are the units of multimeric PROTEINS. Multimeric proteins can be composed of identical or non-identical subunits. One or more monomeric subunits may compose a protomer which itself is a subunit structure of a larger assembly.Cyclic AMP: An adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH.4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid: A non-penetrating amino reagent (commonly called SITS) which acts as an inhibitor of anion transport in erythrocytes and other cells.CHO Cells: CELL LINE derived from the ovary of the Chinese hamster, Cricetulus griseus (CRICETULUS). The species is a favorite for cytogenetic studies because of its small chromosome number. The cell line has provided model systems for the study of genetic alterations in cultured mammalian cells.gamma-Aminobutyric Acid: The most common inhibitory neurotransmitter in the central nervous system.Lipid Bilayers: Layers of lipid molecules which are two molecules thick. Bilayer systems are frequently studied as models of biological membranes.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.HEK293 Cells: A cell line generated from human embryonic kidney cells that were transformed with human adenovirus type 5.Calcium Chloride: A salt used to replenish calcium levels, as an acid-producing diuretic, and as an antidote for magnesium poisoning.Permeability: Property of membranes and other structures to permit passage of light, heat, gases, liquids, metabolites, and mineral ions.G Protein-Coupled Inwardly-Rectifying Potassium Channels: A family of inwardly-rectifying potassium channels that are activated by PERTUSSIS TOXIN sensitive G-PROTEIN-COUPLED RECEPTORS. GIRK potassium channels are primarily activated by the complex of GTP-BINDING PROTEIN BETA SUBUNITS and GTP-BINDING PROTEIN GAMMA SUBUNITS.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Chlorine: A greenish-yellow, diatomic gas that is a member of the halogen family of elements. It has the atomic symbol Cl, atomic number 17, and atomic weight 70.906. It is a powerful irritant that can cause fatal pulmonary edema. Chlorine is used in manufacturing, as a reagent in synthetic chemistry, for water purification, and in the production of chlorinated lime, which is used in fabric bleaching.Potassium Chloride: A white crystal or crystalline powder used in BUFFERS; FERTILIZERS; and EXPLOSIVES. It can be used to replenish ELECTROLYTES and restore WATER-ELECTROLYTE BALANCE in treating HYPOKALEMIA.Hyperpolarization-Activated Cyclic Nucleotide-Gated Channels: A subgroup of cyclic nucleotide-regulated ION CHANNELS of the superfamily of pore-loop cation channels that are opened by hyperpolarization rather than depolarization. The ion conducting pore passes SODIUM, CALCIUM, and POTASSIUM cations with a preference for potassium.KCNQ2 Potassium Channel: A very slow opening and closing voltage-gated potassium channel that is expressed in NEURONS and is commonly mutated in BENIGN FAMILIAL NEONATAL CONVULSIONS.Gold Compounds: Inorganic compounds that contain gold as an integral part of the molecule.Structure-Activity Relationship: The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.Epithelium: One or more layers of EPITHELIAL CELLS, supported by the basal lamina, which covers the inner or outer surfaces of the body.Sequence Homology, Amino Acid: The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.Colforsin: Potent activator of the adenylate cyclase system and the biosynthesis of cyclic AMP. From the plant COLEUS FORSKOHLII. Has antihypertensive, positive inotropic, platelet aggregation inhibitory, and smooth muscle relaxant activities; also lowers intraocular pressure and promotes release of hormones from the pituitary gland.DNA, Complementary: Single-stranded complementary DNA synthesized from an RNA template by the action of RNA-dependent DNA polymerase. cDNA (i.e., complementary DNA, not circular DNA, not C-DNA) is used in a variety of molecular cloning experiments as well as serving as a specific hybridization probe.Mice, Inbred CFTR: A strain of mice widely studied as a model for cystic fibrosis. These mice are generated from embryonic stem cells in which the CFTR (cystic fibrosis transmembrane conductance regulator) gene is inactivated by gene targeting. As a result, all mice have one copy of this altered gene in all their tissues. Mice homozygous for the disrupted gene exhibit many features common to young cystic fibrosis patients, including failure to thrive, meconium ileus, and alteration of mucous and serous glands.Rabbits: The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.Calcium Channels, P-Type: CALCIUM CHANNELS located within the PURKINJE CELLS of the cerebellum. They are involved in stimulation-secretion coupling of neurons.Methylene Chloride: A chlorinated hydrocarbon that has been used as an inhalation anesthetic and acts as a narcotic in high concentrations. Its primary use is as a solvent in manufacturing and food technology.Torpedo: A genus of the Torpedinidae family consisting of several species. Members of this family have powerful electric organs and are commonly called electric rays.Osmolar Concentration: The concentration of osmotically active particles in solution expressed in terms of osmoles of solute per liter of solution. Osmolality is expressed in terms of osmoles of solute per kilogram of solvent.Voltage-Dependent Anion Channels: A family of voltage-gated eukaryotic porins that form aqueous channels. They play an essential role in mitochondrial CELL MEMBRANE PERMEABILITY, are often regulated by BCL-2 PROTO-ONCOGENE PROTEINS, and have been implicated in APOPTOSIS.RNA, Complementary: Synthetic transcripts of a specific DNA molecule or fragment, made by an in vitro transcription system. This cRNA can be labeled with radioactive uracil and then used as a probe. (King & Stansfield, A Dictionary of Genetics, 4th ed)Ammonium Chloride: An acidifying agent that has expectorant and diuretic effects. Also used in etching and batteries and as a flux in electroplating.NAV1.5 Voltage-Gated Sodium Channel: A voltage-gated sodium channel subtype that mediates the sodium ion PERMEABILITY of CARDIOMYOCYTES. Defects in the SCN5A gene, which codes for the alpha subunit of this sodium channel, are associated with a variety of CARDIAC DISEASES that result from loss of sodium channel function.Ligand-Gated Ion Channels: A subclass of ion channels that open or close in response to the binding of specific LIGANDS.Ryanodine Receptor Calcium Release Channel: A tetrameric calcium release channel in the SARCOPLASMIC RETICULUM membrane of SMOOTH MUSCLE CELLS, acting oppositely to SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES. It is important in skeletal and cardiac excitation-contraction coupling and studied by using RYANODINE. Abnormalities are implicated in CARDIAC ARRHYTHMIAS and MUSCULAR DISEASES.Rats, Wistar: A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.Bufo bufo: A species of the true toads, Bufonidae, widely distributed in the United States and Europe.KCNQ3 Potassium Channel: A very slow opening and closing voltage-gated potassium channel that is expressed in NEURONS and is closely related to KCNQ2 POTASSIUM CHANNEL. It is commonly mutated in BENIGN FAMILIAL NEONATAL CONVULSIONS.Cloning, Molecular: The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.Epithelial Cells: Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.Large-Conductance Calcium-Activated Potassium Channel alpha Subunits: The pore-forming subunits of large-conductance calcium-activated potassium channels. They form tetramers in CELL MEMBRANES.Lithium Chloride: A salt of lithium that has been used experimentally as an immunomodulator.Protein Conformation: The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).Guinea Pigs: A common name used for the genus Cavia. The most common species is Cavia porcellus which is the domesticated guinea pig used for pets and biomedical research.Strychnine: An alkaloid found in the seeds of STRYCHNOS NUX-VOMICA. It is a competitive antagonist at glycine receptors and thus a convulsant. It has been used as an analeptic, in the treatment of nonketotic hyperglycinemia and sleep apnea, and as a rat poison.Anion Transport Proteins: Membrane proteins whose primary function is to facilitate the transport of negatively charged molecules (anions) across a biological membrane.Bromides: Salts of hydrobromic acid, HBr, with the bromine atom in the 1- oxidation state. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)Calcium Channels, Q-Type: CALCIUM CHANNELS located in the neurons of the brain.NAV1.2 Voltage-Gated Sodium Channel: A voltage-gated sodium channel subtype that mediates the sodium ion permeability of excitable membranes. Defects in the SCN2A gene which codes for the alpha subunit of this sodium channel are associated with benign familial infantile seizures type 3, and early infantile epileptic encephalopathy type 11.Glycine: A non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter.Trachea: The cartilaginous and membranous tube descending from the larynx and branching into the right and left main bronchi.Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Delayed Rectifier Potassium Channels: A group of slow opening and closing voltage-gated potassium channels. Because of their delayed activation kinetics they play an important role in controlling ACTION POTENTIAL duration.Picrotoxin: A noncompetitive antagonist at GABA-A receptors and thus a convulsant. Picrotoxin blocks the GAMMA-AMINOBUTYRIC ACID-activated chloride ionophore. Although it is most often used as a research tool, it has been used as a CNS stimulant and an antidote in poisoning by CNS depressants, especially the barbiturates.Amiloride: A pyrazine compound inhibiting SODIUM reabsorption through SODIUM CHANNELS in renal EPITHELIAL CELLS. This inhibition creates a negative potential in the luminal membranes of principal cells, located in the distal convoluted tubule and collecting duct. Negative potential reduces secretion of potassium and hydrogen ions. Amiloride is used in conjunction with DIURETICS to spare POTASSIUM loss. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p705)Nephrocalcinosis: A condition characterized by calcification of the renal tissue itself. It is usually seen in distal RENAL TUBULAR ACIDOSIS with calcium deposition in the DISTAL KIDNEY TUBULES and the surrounding interstitium. Nephrocalcinosis causes RENAL INSUFFICIENCY.Calcium Channels, R-Type: CALCIUM CHANNELS located in the neurons of the brain. They are inhibited by the marine snail toxin, omega conotoxin MVIIC.Biophysics: The study of PHYSICAL PHENOMENA and PHYSICAL PROCESSES as applied to living things.Electrophysiological Phenomena: The electrical properties, characteristics of living organisms, and the processes of organisms or their parts that are involved in generating and responding to electrical charges.Protein Binding: The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.Point Mutation: A mutation caused by the substitution of one nucleotide for another. This results in the DNA molecule having a change in a single base pair.Cyclic AMP-Dependent Protein Kinases: A group of enzymes that are dependent on CYCLIC AMP and catalyze the phosphorylation of SERINE or THREONINE residues on proteins. Included under this category are two cyclic-AMP-dependent protein kinase subtypes, each of which is defined by its subunit composition.Gene Expression: The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.Myocardium: The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.Antinematodal Agents: Substances used in the treatment or control of nematode infestations. They are used also in veterinary practice.Sodium-Potassium-Chloride Symporters: A subclass of symporters that specifically transport SODIUM CHLORIDE and/or POTASSIUM CHLORIDE across cellular membranes in a tightly coupled process.Electric Stimulation: Use of electric potential or currents to elicit biological responses.Amino Acid Substitution: The naturally occurring or experimentally induced replacement of one or more AMINO ACIDS in a protein with another. If a functionally equivalent amino acid is substituted, the protein may retain wild-type activity. Substitution may also diminish, enhance, or eliminate protein function. Experimentally induced substitution is often used to study enzyme activities and binding site properties.Barium Compounds: Inorganic compounds that contain barium as an integral part of the molecule.Benzalkonium Compounds: A mixture of alkylbenzyldimethylammonium compounds. It is a bactericidal quaternary ammonium detergent used topically in medicaments, deodorants, mouthwashes, as a surgical antiseptic, and as a as preservative and emulsifier in drugs and cosmetics.Channelopathies: A variety of neuromuscular conditions resulting from MUTATIONS in ION CHANNELS manifesting as episodes of EPILEPSY; HEADACHE DISORDERS; and DYSKINESIAS.Cadmium Chloride: A cadmium halide in the form of colorless crystals, soluble in water, methanol, and ethanol. It is used in photography, in dyeing, and calico printing, and as a solution to precipitate sulfides. (McGraw-Hill Dictionary of Scientific and Technical Terms, 5th ed)Receptors, GABA-A: Cell surface proteins which bind GAMMA-AMINOBUTYRIC ACID and contain an integral membrane chloride channel. Each receptor is assembled as a pentamer from a pool of at least 19 different possible subunits. The receptors belong to a superfamily that share a common CYSTEINE loop.Biophysical Phenomena: The physical characteristics and processes of biological systems.Charybdotoxin: A 37-amino acid residue peptide isolated from the scorpion Leiurus quinquestriatus hebraeus. It is a neurotoxin that inhibits calcium activated potassium channels.Voltage-Gated Sodium Channels: A family of membrane proteins that selectively conduct SODIUM ions due to changes in the TRANSMEMBRANE POTENTIAL DIFFERENCE. They typically have a multimeric structure with a core alpha subunit that defines the sodium channel subtype and several beta subunits that modulate sodium channel activity.Sulfonylurea Receptors: ATP-BINDING CASSETTE PROTEINS that are highly conserved and widely expressed in nature. They form an integral part of the ATP-sensitive potassium channel complex which has two intracellular nucleotide folds that bind to sulfonylureas and their analogs.Muscle, Skeletal: A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.Protein Structure, Secondary: The level of protein structure in which regular hydrogen-bond interactions within contiguous stretches of polypeptide chain give rise to alpha helices, beta strands (which align to form beta sheets) or other types of coils. This is the first folding level of protein conformation.Membrane Transport Modulators: Agents that affect ION PUMPS; ION CHANNELS; ABC TRANSPORTERS; and other MEMBRANE TRANSPORT PROTEINS.Reverse Transcriptase Polymerase Chain Reaction: A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.Bumetanide: A sulfamyl diuretic.Kidney Tubules, Collecting: Straight tubes commencing in the radiate part of the kidney cortex where they receive the curved ends of the distal convoluted tubules. In the medulla the collecting tubules of each pyramid converge to join a central tube (duct of Bellini) which opens on the summit of the papilla.Cattle: Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.Protons: Stable elementary particles having the smallest known positive charge, found in the nuclei of all elements. The proton mass is less than that of a neutron. A proton is the nucleus of the light hydrogen atom, i.e., the hydrogen ion.Nifedipine: A potent vasodilator agent with calcium antagonistic action. It is a useful anti-anginal agent that also lowers blood pressure.Cysteine: A thiol-containing non-essential amino acid that is oxidized to form CYSTINE.Solute Carrier Family 12, Member 2: Na-K-Cl transporter ubiquitously expressed. It plays a key role in salt secretion in epithelial cells and cell volume regulation in nonepithelial cells.Dihydropyridines: Pyridine moieties which are partially saturated by the addition of two hydrogen atoms in any position.Water-Electrolyte Balance: The balance of fluid in the BODY FLUID COMPARTMENTS; total BODY WATER; BLOOD VOLUME; EXTRACELLULAR SPACE; INTRACELLULAR SPACE, maintained by processes in the body that regulate the intake and excretion of WATER and ELECTROLYTES, particularly SODIUM and POTASSIUM.Cadmium: An element with atomic symbol Cd, atomic number 48, and atomic weight 114. It is a metal and ingestion will lead to CADMIUM POISONING.Muscle, Smooth, Vascular: The nonstriated involuntary muscle tissue of blood vessels.Blotting, Western: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.Mutation, Missense: A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)Furosemide: A benzoic-sulfonamide-furan. It is a diuretic with fast onset and short duration that is used for EDEMA and chronic RENAL INSUFFICIENCY.Alprostadil: A potent vasodilator agent that increases peripheral blood flow.Protein Isoforms: Different forms of a protein that may be produced from different GENES, or from the same gene by ALTERNATIVE SPLICING.Receptors, Biogenic Amine: Cell surface proteins that bind biogenic amines with high affinity and regulate intracellular signals which influence the behavior of cells. Biogenic amine is a chemically imprecise term which, by convention, includes the catecholamines epinephrine, norepinephrine, and dopamine, the indoleamine serotonin, the imidazolamine histamine, and compounds closely related to each of these.

Relaxation of endothelin-1-induced pulmonary arterial constriction by niflumic acid and NPPB: mechanism(s) independent of chloride channel block. (1/2847)

We investigated the effects of the Cl- channel blockers niflumic acid, 5-nitro-2-(3-phenylpropylamino)-benzoic acid (NPPB) and 4, 4'-diisothiocyanatostilbene-2,2'-disulphonic acid (DIDS) on endothelin-1 (ET-1)-induced constriction of rat small pulmonary arteries (diameter 100-400 microm) in vitro, following endothelium removal. ET-1 (30 nM) induced a sustained constriction of rat pulmonary arteries in physiological salt solution. Arteries preconstricted with ET-1 were relaxed by niflumic acid (IC50: 35.8 microM) and NPPB (IC50: 21.1 microM) in a reversible and concentration-dependent manner. However, at concentrations known to block Ca++-activated Cl- channels, DIDS (+info)

Chloride dependence of hyperpolarization-activated chloride channel gates. (2/2847)

1. ClC proteins are a class of voltage-dependent Cl- channels with several members mutated in human diseases. The prototype ClC-0 Torpedo channel is a dimeric protein; each subunit forms a pore that can gate independently from the other one. A common slower gating mechanism acts on both pores simultaneously; slow gating activates ClC-0 at hyperpolarized voltages. The ClC-2 Cl- channel is also activated by hyperpolarization, as are some ClC-1 mutants (e.g. D136G) and wild-type (WT) ClC-1 at certain pH values. 2. We studied the dependence on internal Cl- ([Cl-]i) of the hyperpolarization-activated gates of several ClC channels (WT ClC-0, ClC-0 mutant P522G, ClC-1 mutant D136G and an N-terminal deletion mutant of ClC-2), by patch clamping channels expressed in Xenopus oocytes. 3. With all these channels, reducing [Cl-]i shifted activation to more negative voltages and reduced the maximal activation at most negative voltages. 4. We also investigated the external halide dependence of WT ClC-2 using two-electrode voltage-clamp recording. Reducing external Cl- ([Cl-]o) activated ClC-2 currents. Replacing [Cl-]o by the less permeant Br- reduced channel activity and accelerated deactivation. 5. Gating of the ClC-2 mutant K566Q in normal [Cl-]o resembled that of WT ClC-2 in low [Cl-]o, i.e. channels had a considerable open probability (Po) at resting membrane potential. Substituting external Cl- by Br- or I- led to a decrease in Po. 6. The [Cl-]i dependence of the hyperpolarization-activated gates of various ClC channels suggests a similar gating mechanism, and raises the possibility that the gating charge for the hyperpolarization-activated gate is provided by Cl-. 7. The external halide dependence of hyperpolarization-activated gating of ClC-2 suggests that it is mediated or modulated by anions as in other ClC channels. In contrast to the depolarization-activated fast gates of ClC-0 and ClC-1, the absence of Cl- favours channel opening. Lysine 556 may be important for the relevant binding site.  (+info)

Volume regulation following hypotonic shock in isolated crypts of mouse distal colon. (3/2847)

1. A video-imaging technique of morphometry was used to measure the diameter as an index of cell volume in intact mouse distal colon crypts submitted to hypotonic shock. 2. Transition from isotonic (310 mosmol l-1) to hypotonic (240 mosmol l-1) saline caused a pronounced increase in crypt diameter immediately followed by regulatory volume decrease (RVD). 3. Exposure of crypts to Cl--free hyposmotic medium increased the rapidity of both cell swelling and RVD. Exposure of crypts to Na+-free hyposmotic medium reduced the total duration of swelling. Return to initial diameter was followed by further shrinkage of the crypt cells. 4. The chloride channel inhibitor NPPB (50 microM) delayed the swelling phase and prevented the subsequent normal decrease in diameter. 5. The K+ channel blockers barium (10 mM), charybdotoxin (10 nM) and TEA (5 mM) inhibited RVD by 51, 44 and 32 %, respectively. 6. Intracellular [Ca2+] rose from a baseline of 174 +/- 17 nM (n = 8) to 448 +/- 45 nM (n = 8) during the initial swelling phase 7. The Ca2+ channel blockers verapamil (50 microM) and nifedipine (10 microM), the chelator of intracellular Ca2+ BAPTA AM (30 microM), or the inhibitor of Ca2+ release TMB-8 (10 microM), dramatically reduced volume recovery, leading to 51 % (n = 9), 25 % (n = 7), 37 % (n = 6), 32 % (n = 8) inhibition of RVD, respectively. TFP (50 microM), an antagonist of the Ca2+-calmodulin complex, significantly slowed RVD. The Ca2+ ionophore A23187 (2 microM) provoked a dramatic reduction of the duration and amplitude of cell swelling followed by extensive shrinkage. The release of Ca2+ from intracellular stores using bradykinin (1 microM) or blockade of reabsorption with thapsigargin (1 microM) decreased the duration of RVD. 8. Prostaglandin E2 (PGE2, 5 microM) slightly delayed RVD, whereas leukotriene D4 (LTD4, 100 nM) and arachidonic acid (10 microM) reduced the duration of RVD. Blockade of phospholipase A2 by quinacrine (10 microM) inhibited RVD by 53 %. Common inhibition of PGE2 and LTD4 synthesis by ETYA (50 microM) or separate blockade of PGE2 synthesis by 1 microM indomethacin reduced the duration of RVD. Blockade of LTD4 synthesis by nordihydroguaiaretic acid (NDGA) did not produce any significant effect on cell swelling or subsequent RVD. 9. Staurosporine (1 microM), an inhibitor of protein kinases, inhibited RVD by 58 %. Taken together the experiments demonstrate that the RVD process is under the control of conductive pathways, extra- and intracellular Ca2+ ions, protein kinases, prostaglandins and leukotrienes.  (+info)

Acetylcholine-induced membrane potential changes in endothelial cells of rabbit aortic valve. (4/2847)

1. Using a microelectrode technique, acetylcholine (ACh)-induced membrane potential changes were characterized using various types of inhibitors of K+ and Cl- channels in rabbit aortic valve endothelial cells (RAVEC). 2. ACh produced transient then sustained membrane hyperpolarizations. Withdrawal of ACh evoked a transient depolarization. 3. High K+ blocked and low K+ potentiated the two ACh-induced hyperpolarizations. Charybdotoxin (ChTX) attenuated the ACh-induced transient and sustained hyperpolarizations; apamin inhibited only the sustained hyperpolarization. In the combined presence of ChTX and apamin, ACh produced a depolarization. 4. In Ca2+-free solution or in the presence of Co2+ or Ni2+, ACh produced a transient hyperpolarization followed by a depolarization. In BAPTA-AM-treated cells, ACh produced only a depolarization. 5. A low concentration of A23187 attenuated the ACh-induced transient, but not the sustained, hyperpolarization. In the presence of cyclopiazonic acid, the hyperpolarization induced by ACh was maintained after ACh removal; this maintained hyperpolarization was blocked by Co2+. 6. Both NPPB and hypertonic solution inhibited the membrane depolarization seen after ACh washout. Bumetanide also attenuated this depolarization. 7. It is concluded that in RAVEC, ACh produces a two-component hyperpolarization followed by a depolarization. It is suggested that ACh-induced Ca2+ release from the storage sites causes a transient hyperpolarization due to activation of ChTX-sensitive K+ channels and that ACh-activated Ca2+ influx causes a sustained hyperpolarization by activating both ChTX- and apamin-sensitive K+ channels. Both volume-sensitive Cl- channels and the Na+-K+-Cl- cotransporter probably contribute to the ACh-induced depolarization.  (+info)

ATP dependence of the ICl,swell channel varies with rate of cell swelling. Evidence for two modes of channel activation. (5/2847)

Swelling-induced activation of the outwardly rectifying anion current, ICl, swell, is modulated by intracellular ATP. The mechanisms by which ATP controls channel activation, however, are unknown. Whole cell patch clamp was employed to begin addressing this issue. Endogenous ATP production was inhibited by dialyzing N1E115 neuroblastoma cells for 4-5 min with solutions containing (microM): 40 oligomycin, 5 iodoacetate, and 20 rotenone. The effect of ATP on current activation was observed in the absence of intracellular Mg2+, in cells exposed to extracellular metabolic inhibitors for 25-35 min followed by intracellular dialysis with oligomycin, iodoacetate, and rotenone, after substitution of ATP with the nonhydrolyzable analogue AMP-PNP, and in the presence of AMP-PNP and alkaline phosphatase to dephosphorylate intracellular proteins. These results demonstrate that the ATP dependence of the channel requires ATP binding rather than hydrolysis and/or phosphorylation reactions. When cells were swollen at 15-55%/min in the absence of intracellular ATP, current activation was slow (0.3-0.8 pA/pF per min). ATP concentration increased the rate of current activation up to maximal values of 4-6 pA/pF per min, but had no effect on the sensitivity of the channel to cell swelling. Rate of current activation was a saturable, hyperbolic function of ATP concentration. The EC50 for ATP varied inversely with the rate of cell swelling. Activation of current was rapid (4-6 pA/pF per min) in the absence of ATP when cells were swollen at rates >/=65%/min. Intracellular ATP concentration had no effect on current activation induced by high rates of swelling. Current activation was transient when endogenous ATP was dialyzed out of the cytoplasm of cells swollen at 15%/min. Rundown of the current was reversed by increasing the rate of swelling to 65%/min. These results indicate that the channel and/or associated regulatory proteins are capable of sensing the rate of cell volume increase. We suggest that channel activation occurs via ATP-dependent and -independent mechanisms. Increasing the rate of cell swelling appears to increase the proportion of channels activating via the ATP-independent pathway. These findings have important physiological implications for understanding ICl, swell regulation, the mechanisms by which cells sense volume changes, and volume homeostasis under conditions where cell metabolism is compromised.  (+info)

The muscle chloride channel ClC-1 has a double-barreled appearance that is differentially affected in dominant and recessive myotonia. (6/2847)

Single-channel recordings of the currents mediated by the muscle Cl- channel, ClC-1, expressed in Xenopus oocytes, provide the first direct evidence that this channel has two equidistant open conductance levels like the Torpedo ClC-0 prototype. As for the case of ClC-0, the probabilities and dwell times of the closed and conducting states are consistent with the presence of two independently gated pathways with approximately 1.2 pS conductance enabled in parallel via a common gate. However, the voltage dependence of the common gate is different and the kinetics are much faster than for ClC-0. Estimates of single-channel parameters from the analysis of macroscopic current fluctuations agree with those from single-channel recordings. Fluctuation analysis was used to characterize changes in the apparent double-gate behavior of the ClC-1 mutations I290M and I556N causing, respectively, a dominant and a recessive form of myotonia. We find that both mutations reduce about equally the open probability of single protopores and that mutation I290M yields a stronger reduction of the common gate open probability than mutation I556N. Our results suggest that the mammalian ClC-homologues have the same structure and mechanism proposed for the Torpedo channel ClC-0. Differential effects on the two gates that appear to modulate the activation of ClC-1 channels may be important determinants for the different patterns of inheritance of dominant and recessive ClC-1 mutations.  (+info)

A single hydrophobic residue confers barbiturate sensitivity to gamma-aminobutyric acid type C receptor. (7/2847)

Barbiturate sensitivity was imparted to the human rho1 homooligomeric gamma-aminobutyric acid (GABA) receptor channel by mutation of a tryptophan residue at position 328 (Trp328), which is located within the third transmembrane domain. Substitutions of Trp328 with a spectrum of amino acids revealed that nearly all hydrophobic residues produced receptor channels that were both directly activated and modulated by pentobarbital with similar sensitivities. Previous studies with ligand-gated ion channels (including GABA) have demonstrated that even conservative amino acid substitution within the agonist-dependent activation domain (N-terminal extracellular domain) can markedly impair agonist sensitivity. Thus, the lack of significant variation in pentobarbital sensitivity among the Trp328 mutants attests to an intrinsic difference between pentobarbital- and the GABA-dependent activation domain. Compared with the heterooligomeric alphabetagamma receptor channel, the mode of modulation for homooligomeric Trp328 mutants by pentobarbital was more dependent on the GABA concentration, yielding potentiation only at low concentrations of GABA (fractions of their respective EC50 values), yet causing inhibition at higher concentrations. Agonist-related studies have also demonstrated that residue 328 plays an important role in agonist-dependent activation, suggesting a functional interconnection between the GABA and pentobarbital activation domains.  (+info)

Functional and molecular characterization of a volume-sensitive chloride current in rat brain endothelial cells. (8/2847)

1. Volume-activated chloride currents in cultured rat brain endothelial cells were investigated on a functional level using the whole-cell voltage-clamp technique and on a molecular level using the reverse transcriptase-polymerase chain reaction (RT-PCR). 2. Exposure to a hypotonic solution caused the activation of a large, outward rectifying current, which exhibited a slight time-dependent decrease at strong depolarizing potentials. The anion permeability of the induced current was I- (1.7) > Br- (1.2) > Cl- (1.0) > F- (0. 7) > gluconate (0.18). 3. The chloride channel blocker 5-nitro-2-(3-phenylpropylamino)-benzoate (NPPB, 100 microM) rapidly and reversibly inhibited both inward and outward currents. The chloride transport blocker 4,4'-diisothiocyanatostilbene-2, 2'-disulphonic acid (DIDS, 100 microM) also blocked the hypotonicity-induced current in a reversible manner. In this case, the outward current was more effectively suppressed than the inward current. The volume-activated current was also inhibited by the antioestrogen tamoxifen (10 microM). 4. The current was dependent on intracellular ATP and independent of intracellular Ca2+. 5. Activation of protein kinase C by phorbol 12,13-dibutyrate (PDBu, 100 nM) inhibited the increase in current normally observed following hypotonic challenge. 6. Extracellular ATP (10 mM) inhibited the current with a more pronounced effect on the outward than the inward current. 7. Verapamil (100 microM) decreased both the inward and the outward hypotonicity-activated chloride current. 8. RT-PCR analysis was used to determine possible molecular candidates for the volume-sensitive current. Expression of the ClC-2, ClC-3 and ClC-5 chloride channels, as well as pICln, could be shown at the mRNA level. 9. We conclude that rat brain endothelial cells express chloride channels which are activated by osmotic swelling. The biophysical and pharmacological properties of the current show strong similarities to those of ClC-3 channel currents as described in other cell types.  (+info)

*Chloride channel

Chloride channels are a superfamily of poorly understood ion channels specific for chloride. These channels may conduct many ... The chloride channel subunits contain between 1 and 12 transmembrane segments. Some chloride channels are activated only by ... Based on sequence homology the chloride channels can be subdivided into a number of groups. Voltage-gated chloride channels are ... CLC chloride channels As of this edit, this article uses content from "1.A.13 The Epithelial Chloride Channel (E-ClC) Family", ...

*Chloride channel opener

A chloride channel opener is a type of drug which facilitates ion transmission through chloride channels. An example is 1,10- ... Chloride channel blocker Duszyk M, MacVinish L, Cuthbert AW (2001). "Phenanthrolines--a new class of CFTR chloride channel ...

*Chloride channel blocker

A chloride channel blocker is a type of drug which inhibits the transmission of ions (Cl−) through chloride channels. Niflumic ... acid is a chloride channel blocker that has been used in experimental scientific research. Chloride channel opener Knauf PA, ...

*Calcium-dependent chloride channel

... chloride channel accessory (CLCA), bestrophin (BEST), and calcium-dependent chloride channel anoctamin (ANO or TMEM16) channels ... The Calcium-Dependent Chloride Channel (Ca-ClC) proteins (or calcium-activated chloride channels (CaCCs), are heterogeneous ... "1.A.17 The Calcium-Dependent Chloride Channel (Ca-ClC) Family". TCDB. Retrieved 2016-04-16. "Calcium activated chloride channel ... and BEST4 Voltage-gated ion channel Ion channel Calcium-activated chloride channel Transporter Classification Database "TCDB » ...

*CLCN6

Chloride channel 6 and 7 belong to a subbranch of this family. Chloride channel 6 has four different alternatively spliced ... This gene is in close vicinity to two other kidney-specific chloride channel genes, CLCNKA and CLCNKB. Chloride channel GRCh38 ... "Entrez Gene: CLCN6 chloride channel 6". Nakajima D, Okazaki N, Yamakawa H, et al. (2003). "Construction of expression-ready ... The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite ...

*CLCNKA

... encodes one of the two major chloride channels found in the kidney, the ClC-Ka channel (the other class being the ClC-Kb ... "Entrez Gene: CLCNKA chloride channel Ka". Barlassina C, Dal Fiume C, Lanzani C, Manunta P, Guffanti G, Ruello A, Bianchi G, Del ... Chloride channel protein ClC-Ka is a protein that in humans is encoded by the CLCNKA gene. Multiple transcript variants ... The Arg83Gly SNP specifically results in a large reduction in the flow of chloride ions through the ClC-Ka channel in the thin ...

*Dent's disease

These chloride channels have an important role in the control of membrane excitability, transepithelial transport, and possibly ... CLCN5 belongs to the family of voltage-gated chloride channel genes (CLCN1-CLCN7, CLCKa and CLCKb) that have about 12 ... Jentsch TJ, Friedrich T, Schriever A, Yamada H (May 1999). "The CLC chloride channel family". Pflugers Arch. 437 (6): 783-95. ... August 2000). "Characterization of renal chloride channel (CLCN5) mutations in Dent's disease". J. Am. Soc. Nephrol. 11 (8): ...

*CLCN7

Chloride channel 7 alpha subunit also known as H+/Cl− exchange transporter 7 is a protein that in humans is encoded by the ... 2004). "Chloride channel 7 (ClCN7) gene mutations and autosomal dominant osteopetrosis, type II". J. Bone Miner. Res. 18 (8): ... Brandt S, Jentsch TJ (1996). "ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family". FEBS ... 2001). "Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man". Cell. 104 (2): 205-15. doi:10.1016/S0092- ...

*CLCN4

Chloride channel 4 has an evolutionary conserved CpG island and is conserved in both mouse and hamster. This gene is mapped in ... "Entrez Gene: CLCN4 chloride channel 4". Veeramah KR, Johnstone L, Karafet TM, Wolf D, Sprissler R, Salogiannis J, Barth-Maron A ... Lamb FS, Clayton GH, Liu BX, Smith RL, Barna TJ, Schutte BC (1999). "Expression of CLCN voltage-gated chloride channel genes in ... The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite ...

*CLIC3

This protein is a chloride channel. Chloride channel GRCh38: Ensembl release 89: ENSG00000169583 - Ensembl, May 2017 GRCm38: ... Chloride intracellular channel protein 3 is a protein that in humans is encoded by the CLIC3 gene. ... 1997). "Molecular cloning and expression of a chloride ion channel of cell nuclei". J. Biol. Chem. 272 (19): 12575-82. doi: ... Suginta W, Karoulias N, Aitken A, Ashley RH (2001). "Chloride intracellular channel protein CLIC4 (p64H1) binds directly to ...

*CLCN2

2006). "Association between Hsp90 and the ClC-2 chloride channel upregulates channel function". Am. J. Physiol., Cell Physiol. ... CLCN2 chloride channel 2". Haug K, Warnstedt M, Alekov AK, et al. (2003). "Mutations in CLCN2 encoding a voltage-gated chloride ... Chloride channel protein 2 is a protein that in humans is encoded by the CLCN2 gene. Mutations of this gene have been found to ... 2004). "Is the ClC-2 chloride channel involved in the Cl− secretory mechanism of gastric parietal cells?". FEBS Lett. 575 (1-3 ...

*CLCN3

... chloride channel 3". Gentzsch M, Cui L, Mengos A, Chang XB, Chen JH, Riordan JR (Feb 2003). "The PDZ-binding chloride ... Gentzsch M, Cui L, Mengos A, Chang XB, Chen JH, Riordan JR (2003). "The PDZ-binding chloride channel ClC-3B localizes to the ... Lamb FS, Clayton GH, Liu BX, Smith RL, Barna TJ, Schutte BC (1999). "Expression of CLCN voltage-gated chloride channel genes in ... Lamb FS, Graeff RW, Clayton GH, Smith RL, Schutte BC, McCray PB (2001). "Ontogeny of CLCN3 chloride channel gene expression in ...

*Avermectin

Bloomquist, Jeffrey R. (2003). "Chloride channels as tools for developing selective insecticides". Archives of Insect ... comparable doses are not toxic for mammals because they do not possess glutamate-gated chloride channels. Resistance to ... mode of action and target site-mediated resistance to insecticides acting on chloride channels". Comparative Biochemistry and ... "Cloning of an avermectin-sensitive glutamate-gated chloride channel from Caenorhabditis elegans". Nature. 371 (6499): 707-11. ...

*Tweety family member 2

The encoded protein functions as a calcium(2+)-activated large conductance chloride(-) channel, and may play a role in kidney ... Members of this family function as chloride channels. ... of the human Tweety family of putative chloride ion channels ... ligase Nedd4-2 differentially interacts with and regulates members of the Tweety family of chloride ion channels". The Journal ... Suzuki M, Mizuno A (May 2004). "A novel human Cl(-) channel family related to Drosophila flightless locus". The Journal of ...

*Tetratricopeptide repeat protein 39B

TTC39B is surrounded by LOC100419056, a chloride channel, voltage-sensitive 3 pseudogene. TTC39B is expected to have a ... "LOC100419056 chloride channel, voltage-sensitive 3 pseudogene". NCBI. Retrieved 13 May 2013. "TTC39B, a comprehensive ... TPR motifs that are arranged one in front of another create a right-handed helical structure with an amphipathic channel which ...

*ANO1

... is a transmembrane protein that functions as a calcium-activated chloride channel. Ca2+, Sr2+, and Ba2+ activate the ... Anoctamin-1 is a voltage-sensitive calcium-activated chloride channel that is expressed in smooth muscle and epithelial cells; ... Ni YL, Kuan AS, Chen TY (2014). "Activation and Inhibition of TMEM16A Calcium-Activated Chloride Channels". PLoS ONE. 9 (1): ... No atomic resolution structure of this channel has yet been obtained. However, biochemical evidence suggests that the channel ...

*Magnesium transporter

They are found in several voltage-gated chloride channels. The orientation in the membrane and the positions of the N- and C- ... Ponting CP (Mar 1997). "CBS domains in CIC chloride channels implicated in myotonia and nephrolithiasis (kidney stones)". ... TRPM6 channel. The report presents a robust collection of data consistent with a channel-like activity passing Mg2+, based on ... The results suggested that Alr1p is very likely to act as an ion-selective channel. In the same paper, the authors reported ...

*Cystic fibrosis transmembrane conductance regulator

Gentzsch M, Cui L, Mengos A, Chang XB, Chen JH, Riordan JR (2003). "The PDZ-binding chloride channel ClC-3B localizes to the ... Cormet-Boyaka E, Di A, Chang SY, Naren AP, Tousson A, Nelson DJ, Kirk KL (2002). "CFTR chloride channels are regulated by a ... The CFTR gene codes for an ABC transporter-class ion channel protein that conducts chloride and thiocyanate ions across ... Mutations of the CFTR gene affecting chloride ion channel function lead to dysregulation of epithelial fluid transport in the ...

*CLNS1A

"Entrez Gene: CLNS1A chloride channel, nucleotide-sensitive, 1A". Larkin D, Murphy D, Reilly DF, Cahill M, Sattler E, Harriott P ... Schwartz RS, Rybicki AC, Nagel RL (1997). "Molecular cloning and expression of a chloride channel-associated protein pICln in ... Chloride channel GRCh38: Ensembl release 89: ENSG00000074201 - Ensembl, May 2017 GRCm38: Ensembl release 89: ENSMUSG00000025439 ... coding for the swelling-dependent chloride channel ICln". Genomics. 38 (3): 438-41. doi:10.1006/geno.1996.0651. PMID 8975725. " ...

*Channel-conductance-controlling ATPase

Sheppard DN, Welsh MJ (1999). "Structure and function of the CFTR chloride channel". Physiol. Rev. 79 (1 Suppl): S23-45. PMID ... In enzymology, a channel-conductance-controlling ATPase (EC 3.6.3.49) is an enzyme that catalyzes the chemical reaction ATP + ... The systematic name of this enzyme class is ATP phosphohydrolase (channel-conductance-controlling). As of late 2007, two ...

*Onchocerciasis

The drug binds to and activates glutamate-gated chloride channels. These channels, present in neurons and myocytes, are not ... Wolstenholme AJ, Rogers AT (2005). "Glutamate-gated chloride channels and the mode of action of the avermectin/milbemycin ... Yates DM, Wolstenholme AJ (August 2004). "An ivermectin-sensitive glutamate-gated chloride channel subunit from Dirofilaria ... Ivermectin is thought to irreversibly activate these channel receptors in the worm, eventually causing an inhibitory ...

*Glutamate (neurotransmitter)

Avermectins target the alpha subunit of glutamate-gated chloride channels with high affinity. These receptors have also been ... In arthropods and nematodes, glutamate stimulates glutamate-gated chloride channels.[citation needed] The β subunits of the ... and glutamate-gated chloride channel (GluCl) alpha receptor subunits in sea lice (Lepeophtheirus salmonis)". Journal of ... "Cloning of an avermectin-sensitive glutamate-gated chloride channel from Caenorhabditis elegans". Nature. 371 (6499): 707-11. ...

*STX1A

Cormet-Boyaka E, Di A, Chang SY, Naren AP, Tousson A, Nelson DJ, Kirk KL (September 2002). "CFTR chloride channels are ... "Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms". Nature. 390 (6657): 302-5. doi:10.1038/36882. PMID ... "Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms". Nature. 390 (6657): 302-5. doi:10.1038/36882. PMID ... Syntaxin-1A is a key protein in ion channel regulation and synaptic exocytosis. Syntaxins serve as a substrate for botulinum ...

*Anion-conducting channelrhodopsin

... ligand-gated chloride channels found in inhibitory synapses: As the chloride concentration in mature neurons is very low, ... When expressed in nerve cells, anion-conducting channelrhodopsins act as light-gated chloride channels. Their effect on the ... As the main anion of extracellular fluid is chloride (Cl−), anion-conducting channelrhodopsins are also known as "chloride- ... "Conversion of Channelrhodopsin into a Light-Gated Chloride Channel". Science. 344 (6182): 409-412. doi:10.1126/science.1249375 ...

*Channelrhodopsin

... charged amino acid arginine turns channelrhodopsin from an unspecific cation channel into a chloride-conducting channel (ChloC ... "Conversion of Channelrhodopsin into a light-gated chloride channel". Science. 344: 409-412. doi:10.1126/science.1249375. PMID ... Closing of the channel after optical activation can be substantially delayed by mutating the protein residues C128 or D156. ... Recently, chloride-conducting channelrhodopsins have been engineered and were also found in nature. These tools can be used to ...

*Halobacterium salinarum

Reilly, Michael; The Discovery Channel. "World's oldest known DNA discovered". Retrieved 3 September 2010. Park, J. S.; ... in particular potassium chloride) to reduce osmotic stress. Potassium levels are not at equilibrium with the environment, so H ...
... , Authors: Velayuthan C Padmakumar, Stuart H Yuspa. Published in: Atlas Genet Cytogenet Oncol Haematol.
AbstractBackground and Aim:Our previous proteomic research found that chloride intracellular channel 1 (CLIC1) was upregulated in hepatocellular carcinoma (HCC) tissues with portal vein tumor thrombus. The present study aimed to determine the role of CLIC1 in HCC invasion.Methods:Immunohistochemistr
Chloride channels are a superfamily of poorly understood ion channels specific for chloride. These channels may conduct many different ions, but are named for chloride because its concentration in vivo is much higher than other anions. Several families of voltage-gated channels and ligand-gated channels (e.g., the CaCC families) have been characterized in humans. Voltage-gated chloride channels display a variety of important physiological and cellular roles that include regulation of pH, volume homeostasis, organic solute transport, cell migration, cell proliferation and differentiation. Based on sequence homology the chloride channels can be subdivided into a number of groups. Voltage-gated chloride channels are important for setting cell resting membrane potential and maintaining proper cell volume. These channels conduct Cl− as well as other anions such as HCO− 3, I−, SCN−, and NO− 3. The structure of these channels are not like other known channels. The chloride channel subunits ...
0017] Also provided herein is a method of identifying an agent that is an inhibitor of a calcium-activated chloride channel comprising: (a) contacting a cell and a candidate agent in a test sample, (under conditions and for a time sufficient) to permit interaction between the candidate agent and the cell, wherein the cell comprises (i) a calcium-activated chloride channel and (ii) a cytoplasmic indicator protein that binds halide; (b) adding to the test sample (i) at least one calcium-elevating agonist and (ii) iodide, under conditions and for a time sufficient for the calcium-elevating agonist to bind to the cell (i.e., to permit binding of the calcium-elevating agonist to the cell), wherein binding of the calcium-elevating agonist to the cell increases the level of calcium ion (Ca2+) in the cell; and (c) determining the level of iodide influx in the presence of the candidate agent and comparing the level of iodide influx in the presence of the candidate agent with the level of iodide influx in ...
Two new calcium-activated chloride channel (CLCA) family members, mCLCA5 and mCLCA6, have been cloned from mouse eye and intestine, respectively. mCLCA5 is highly homologous to hCLCA2, and mCLCA6 is highly homologous to hCLCA4. mCLCA5 is widely expressed with strong expression in eye and spleen, whe …
RefSeq Summary (NM_001288): Chloride channels are a diverse group of proteins that regulate fundamental cellular processes including stabilization of cell membrane potential, transepithelial transport, maintenance of intracellular pH, and regulation of cell volume. Chloride intracellular channel 1 is a member of the p64 family; the protein localizes principally to the cell nucleus and exhibits both nuclear and plasma membrane chloride ion channel activity. [provided by RefSeq, Jul 2008 ...
Read "Permeant Anions Control Gating of Calcium-dependent Chloride Channels, The Journal of Membrane Biology" on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips.
CLIC1 antibody, N-term (chloride intracellular channel 1) for IHC-P, WB. Anti-CLIC1 pAb (GTX48722) is tested in Human, Mouse samples. 100% Ab-Assurance.
Anoctamins and Bestrophins are two kinds of calcium-activated chloride channels. Anoctamins were shown to be Ca-activated Cl channels in 2008 and are responsible for the classical CaCCs that are widely expressed in many tissues, especially epithelia. Bestrophins are more restricted in their expression and play more specialized roles. Bestrophin-1 is expressed in the retinal pigment epithelium and certain mutations in bestrophin-1 cause degeneration of the retina and blindness. Bestrophin-2 is expressed in goblet cells in colon and may play a role in bicarbonate secretion in this tissue. We are interested in understanding how both anoctamins and bestrophins are regulated by calcium. We are identifying the calcium binding sites and the molecular links between the calcium sensor and the channel gate using a combination of both whole-cell and single channel electrophysiological mesurements and mutagenesis. We are dissecting the signaling protein network associated with these channels using ...
Plasma membrane Cl- channels perform a variety of functions, including control of excitability in neurons and muscle, cell volume regulation and transepithelial transport. Structurally, three classes of Cl- channels have been identified: ligand-gated, postsynaptic Cl- channels (e.g. GABA and glycine receptors); the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels (which belong to the traffic ATPase superfamily); and the CLC family of Cl- channels. Recent developments of note include further characterization of the expanding CLC Cl- channel family, advances in understanding the regulation of the CFTR Cl- channel and its emergent role as a regulator of other channels, clarification of issues related to swelling-activated Cl- channels, and the discovery that several co-transporter molecules are now known to induce Cl- currents in Xenopus oocytes.. ...
Proteins encoded by best1 to -3 genes are implicated as molecular correlates of calcium-activated chloride channels in epithelia. In this issue of Circulation Research, Matchkov et al present compelling evidence that best-3 expression is essential for the generation of calcium-sensitive cGMP-dependent chloride channels in rat mesenteric artery.1. Chloride channels are enigmatic beasts. Numerous phenotypes exist, as determined by their mode of activation, channel kinetics, and pore properties, but the molecular identity has only really been identified for the voltage-dependent Cl− channels (CLCs) and the cAMP-dependent, cystic fibrosis transmembrane regulator (CFTR) channels. For the other types of Cl− channels, there is far less certainty about the molecular identity. Many candidates for the swelling-activated Cl− channel have been promulgated that ultimately have been repudiated.2 Similarly, the molecular identity of the Ca2+-gated Cl− channel, common to vascular smooth muscle cells, ...
May be involved in mediating calcium-activated chloride conductance (PubMed:8537359). May play critical roles in goblet cell metaplasia, mucus hypersecretion, cystic fibrosis and AHR. May be involved in the regulation of mucus production and/or secretion by goblet cells. Involved in the regulation of tissue inflammation in the innate immune response. May play a role as a tumor suppressor. Induces MUC5AC.
High blood pressure is the leading risk factor for death worldwide. One of the hallmarks is a rise of peripheral vascular resistance, which largely depends on arteriole tone. Ca2+-activated chloride currents (CaCCs) in vascular smooth muscle cells (VSMCs) are candidates for increasing vascular contractility. We analyzed the vascular tree and identified substantial CaCCs in VSMCs of the aorta and carotid arteries. CaCCs were small or absent in VSMCs of medium-sized vessels such as mesenteric arteries and larger retinal arterioles. In small vessels of the retina, brain, and skeletal muscle, where contractile intermediate cells or pericytes gradually replace VSMCs, CaCCs were particularly large. Targeted disruption of the calcium-activated chloride channel TMEM16A, also known as ANO1, in VSMCs, intermediate cells, and pericytes eliminated CaCCs in all vessels studied. Mice lacking vascular TMEM16A had lower systemic blood pressure and a decreased hypertensive response following vasoconstrictor ...
TY - JOUR. T1 - Characterization of a native swelling-induced chloride current, ICl.swell, and its regulatory protein, pICln, in Xenopus oocytes.. AU - Ackerman, Michael John. AU - Krapivinsky, G. B.. AU - Gordon, E.. AU - Krapivinsky, L.. AU - Clapham, D. C.. PY - 1994. Y1 - 1994. N2 - The ability to precisely regulate cell volume is a fundamental property of most cells. Although the phenomenon of regulatory volume decrease (RVD), whereby a swollen cell loses salt and water to restore its original volume, has been appreciated for decades, the molecular identities of the proteins responsible for the volume control machinery and their regulation are essentially unknown. It appears that the rate-determining step in gaining volume control involves the activation of potassium and chloride conductance pathways. We have identified a native chloride current (ICl.swell) responsive to cell swelling in Xenopus oocytes [Ackerman et al. (1994) J Gen Physiol 103: 153-179]. Moreover, we have demonstrated that ...
The CLC family of chloride channels and transporters is made up by nine members but just three of these ClC-Ka/b ClC-7 and ClC-2 have already been found up to now connected with auxiliary subunits. immunoglobulin (Ig)-like domains regulates its subcellular localization and activity in glial cells. The normal theme for these three proteins can be their requirement of an effective homeostasis since their breakdown leads to specific illnesses. We will review right here their properties and their part in regular chloride physiology as well as the pathological outcomes of their incorrect function. Intro Chloride is very important to many biological features such Apremilast as for example transepithelial fluid transportation acidification of intracellular organelles muscle tissue contraction neuronal membrane potential or cell quantity rules. Chloride flux across membranes is mediated by several classes of proteins (Duran oocytes or in transfected cells (Steinmeyer gene lead to classical Bartter ...
Miller, A. N.*, Vaisey, G.*, & Long, S. B. (2019). Molecular mechanisms of gating in the calcium-activated chloride channel bestrophin. eLife (Abstract , open state Cryo-EM map , Atomic Coordinates). *A. N. Miller and G. Vaisey are co-first authors.. Vaisey, G., & Long, S. B. (2018). An allosteric mechanism of inactivation in the calcium-dependent chloride channel BEST1. The Journal of General Physiology, jgp.201812190. (Abstract). Hou, X., Burstein, S. R., & Long, S. B. (2018). Structures reveal opening of the store-operated calcium channel Orai. eLife, 7. (Manuscript , Atomic Coordinates). Baradaran, R., Wang, C., Siliciano, A. F., & Long, S. B. (2018). Cryo-EM structures of fungal and metazoan mitochondrial calcium uniporters. Nature, 559(7715), 580-584. (Abstract , Atomic Coordinates , Cryo-EM map). Melinda Diver, Leanne Pedi, Akiko Koide, Shohei Koide, Stephen B. Long (2018). Atomic structure of the eukaryotic intramembrane RAS methyltransferase ICMT. Nature. Jan 25; 553(7689):526-529. ...
Furthermore, the inhibitor pharmacology of the VACC also confirms that they are VACCs. Conventional chloride channel blockers such as NPPB and DIDS significantly inhibited the volume-activated currents, indicating that they are chloride channels (21). In various cell types, DIDS has been shown to inhibit VACCs in voltage-dependent fashion (26, 32, 33) in addition to inhibiting calcium-activated chloride channels (1, 3), outwardly rectifying chloride channels (5), and ATP release (5) but does not inhibit CFTR (1, 10). Thus the voltage-dependent inhibition of volume-activated current by DIDS implies that chloride conductance via CFTR may not play a major role in RVD in cholangiocytes, and this conclusion is consistent with the previous finding that cAMP agonists had no significant effect on RVD in cholangiocytes (6). The VACCs are also inhibited by glybenclamide (Fig. 5C), which is a known inhibitor of CFTR, VACCs, as well as ATP-sensitive potassium channels (27). Moreover, the significant ...
Superfusion of BC3H1 cells with the normal hyposmotic solution caused cell swelling accompanied by a large increase in membrane currents in all the tested cells (Fig. 1 A). Membrane currents returned to basal level after switching back to isosmotic solution (Voets et al., 1997). Current-voltage relations reconstructed from voltage ramps applied during the hyposmotic challenge (Fig. 1 B) show that the swelling-activated current reverses close to ECl (−20 mV). We have previously shown that this current is a Cl− current, ICl,swell, with an I− , Br− , Cl− ,, gluconate anion permeability sequence and that it is efficiently blocked by niflumic acid, flufenamic acid, and NPPB (Voets et al., 1997). This current inactivates during voltage steps to positive potentials ,+40 mV (Fig. 1 C). The negative slope of the current-voltage relation seen at very positive potentials (Fig. 1 B) is due to inactivation of the current during the slow voltage ramp.. We observed single-channel Cl− currents in ...
Ion channels catalyze the transportation of small ions across the cell membrane and, in doing so, maintain normal cellular functions. To achieve functional flexibility, these membrane proteins are regulated by a variety of factors, such as transmembrane voltages, mechanical stretches, extracellular or intracellular ligands, and sometimes the small ions in the aqueous solution (Hille 1992). In vertebrate skeletal muscles, for instance, external Cl− and H+ significantly influence the resting membrane conductance and, thus, control the firing pattern of action potentials (Hodgkin and Horowicz 1959, Hodgkin and Horowicz 1960). Electrophysiological recordings and flux assays have shown that the muscle membrane is more permeable to Cl− than to K+ (Hodgkin and Horowicz 1959; Hutter and Padsha 1959; Hutter and Noble 1960), and if the Cl− conductance is removed, the membrane current shows little change when the pH is altered (Hutter and Warner 1967, Hutter and Warner 1972). These early studies ...
Oxyntic cells that retain distinct morphological polarity between apical and basolateral membranes were isolated from the gastric mucosa of the amphibian Necturus. Patch-clamp techniques were applied to these cells to identify apical membrane ion channels associated with hydrochloric acid secretion. A single class of voltage-dependent, inwardly rectifying chloride channels was observed in the apical membranes of both resting and stimulated (acid-secreting) oxyntic cells. Stimulation of the cells with dibutyryladenosine 3,5-monophosphate and isobutylmethylxanthine increased channel open probability and simultaneously increased apical membrane surface area. This chloride channel is probably responsible for electrogenic chloride secretion by the gastric mucosa and may also participate in the fluid- and enzyme-secretory functions of the oxyntic cell, analogous to the chloride channels found in the apical membranes of other exocrine cells. ...
In mature mammalian muscle, the chloride conductance of the membrane is an important factor in the regulation of excitability. Up to now, no ligand was available for the biochemical characterization of muscle chloride channels. In order to localize and characterize these channels, we have used indanyloxyacetic acid (IAA)-94, a ligand previously used for epithelial Cl- channels (Landry, D. W., Reitman, M., Cragoe, E. J., Jr., and Al-Awqati, Q. (1987) J. Gen. Physiol. 90, 779-798; Landry, D. W., Akabas, M. H., Redhead, C., Edelman, A., Cragoe, E. J., Jr., and Al-Awqati, Q. (1989) Science 244, 1469-1472). IAA induced myotonic responses when microinjected into mature mouse muscle fibers, indicating a blockade of Cl- channels from the cytoplasmic side. Membrane vesicles were prepared from rabbit skeletal muscle and separated by sucrose gradient centrifugation. Fractions obtained (in the order of increasing density) were sarcolemma (SL), T-tubules (TT), sarcoplasmatic reticulum (SR), and triads and ...
Shikonin is a major component of a Chinese herbal medicine named zicao. Shikonin is a potent TMEM16A chloride channel inhibitor with an IC50 of 6.5 μM. Shikonin is a specific pyruvate kinase M2 (PKM2) inhibitor and can also inhibit TNF-α and NF-κB pathway. - Mechanism of Action & Protocol.
Calcium-activated chloride channel (CaCC) which plays an important role in transepithelial anion transport and smooth muscle contraction. Required for the normal functioning of the interstitial cells of Cajal (ICCs) which generate electrical pacemaker activity in gastrointestinal smooth muscles. Acts as a major contributor to basal and stimulated chloride conductance in airway epithelial cells and plays an important role in tracheal cartilage development.
Dr. Lily Jan defines the physiological role of ion channels, like potassium channels and Calcium-Activated Chloride Channels, in the regulation of excitability in neurons.
Fusion protein amino acids 568-746 (C-terminus) of rat Clcn5 (also known as H(+)/Cl(-) exchange transporter 5, Chloride channel protein 5, Chloride transporter ClC-5, Clc5 and CLCK2, accession number P51796)Mouse: 99% identity (178/179 amino acids identical)Human: 98% identity (177/179 amino acids identical)70% identit
Catalysis of facilitated diffusion of a chloride (by an energy-independent process) involving passage through a transmembrane aqueous pore or channel without evidence for a carrier-mediated mechanism.
Myotonia is a state of hyperexcitability of skeletal-muscle fibres. Mutations in the ClC-1 Cl− channel cause recessive and dominant forms of this disease. Mutations have been described throughout the protein-coding region, including three sequence variations (A885P, R894X and P932L) in a distal C-terminal stretch of residues [CTD (C-terminal domain) region] that are not conserved between CLC proteins. We show that surface expression of these mutants is reduced in Xenopus oocytes compared with wild-type ClC-1. Functional, biochemical and NMR spectroscopy studies revealed that the CTD region encompasses a segment conserved in most voltage-dependent CLC channels that folds with a secondary structure containing a short type II poly-proline helix. We found that the myotonia-causing mutation A885P disturbs this structure by extending the poly-proline helix. We hypothesize that this structural modification results in the observed alteration of the common gate that acts on both pores of the channel. ...
Cystic fibrosis occurs due to mutation of the chromosome 7 which lead to defect in the chloride ion channel membrane ( CFTR). The common form of mutation is the ΔF508. The defect in the chloride ion channel membrane may lead to the defect in the trans
TY - JOUR. T1 - Phylogenetic shadowing of a histamine-gated chloride channel involved in insect vision. AU - Iovchev, M. AU - Boutanaev, A. AU - Ivanov, I. AU - Wolstenholme, A. AU - Nurminsky, D. AU - Semenov, E. N1 - ID number: ISI:000234767300002. PY - 2006. Y1 - 2006. N2 - A recently identified gene, hlcA (synonym: ort), codes for an ionotrophic histamine receptor Subunit in Drosophila melanogaster, and known hclA mutations lead to defects in the Visual system, neurologic disorders and changed responsiveness to neurotoxins. To investigate whether this novel class of receptors is common across the Insecta, we analysed the genomes of 15 other insect species (Diptera, Hymenoptera, Coleoptera, Lepidoptera) and revealed orthologs of hclA in all of them. The predicted receptor domain of HCLA is extensively conserved (86-100% of identity) among the 16 proteins. Minor changes in the amino acid sequence that includes the putative transmembrane domains (TMs) 1-3 were found in non-drosophilid species ...
Compare chloride channel 6 ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, and more.
Compare chloride channel 6 ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, and more.
A - Tilt: 12° - Segments: 1(36-65), 2(79-100), 3(126-141), 4(148-159), 5(179-190), 6(193-204), 7(215-232), 8(252-280), 9(288-307), 10(333-350), 11(357-372), 12(386-402), 13(405-416), 14(422-438 ...
Since anoctamin 1 ANO1 (TMEM16A) was found to be a molecular component of Ca(2+) -activated Cl(-) channels, its role in tumorigenesis has gained attention at a fast pace. ANO1 overexpression frequently occurs in the cancer tissues along with 11q13 chromosome amplification. Poor prognosis of many types of cancers has been closely correlated with ANO1 gene amplification and protein overexpression. ANO1 is now considered an excellent biomarker for certain cancers. Recent research suggests that it is the channel function of ANO1 that is involved in the tumorigenesis. However, how the overexpression of the functional ANO1 causes malignant transformation of tissues via signaling pathways, for example, MAPK remains to be investigated. Clarification of the reasons in future will avail to make ANO1 as a target for cancer treatment ...
View mouse Clcnka Chr4:141384610-141398724 with: phenotypes, sequences, polymorphisms, proteins, references, function, expression
We are delighted to share with you the latest episode of our Behind the Scenes series. Within this series, we explore the inner workings of the Consortium with a camera in hand, dedicating to showing you what takes place within the laboratory setting and outside of it.. For our second feature, we present the following video feature of Emad Tajkhorshid, an active participant in the Consortiums Computational Modeling Core and the Structural Dynamics of ABC Transporter and Conformational Dynamics in the CLC Channel/Transporter Family projects. We sat down with Emad and asked him about his work with computational modeling, how his laboratory collaborates with other entities within the Consortium, and how he views the role of the Consortium within the broader scientific community. Let us know what you think in the comments!. ...
Complete information for ANO1 gene (Protein Coding), Anoctamin 1, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Complete information for ANO4 gene (Protein Coding), Anoctamin 4, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
韩国CLC女团,2015年3月出道,备受期待。CLC成员共5人,分别是吴承姬、崔有真、张丞延、Sorn、张睿恩。CLC是CrystaLClear的缩写,寓意成员们能像水晶一样永远保持在洁净、闪耀的模样。CLC组合早在出道前就经常在街头演出,积累经验。CLC组合中文网将会汇集CLC的新闻,以最快的速度报道CLC最全面的信息。
TMEM204兔多克隆抗体(ab113102)可与小鼠, 大鼠, 人样本反应并经WB, IHC, ICC, ICC/IF实验严格验证。所有产品均提供质保服务,中国75%以上现货。
Tmem18兔多克隆抗体(ab106597)可与小鼠, 大鼠, 人样本反应并经WB, IHC, ICC/IF实验严格验证。所有产品均提供质保服务,中国75%以上现货。
BACKGROUND: Dents disease, an X-linked renal tubular disorder, is characterized by low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis, and progressive renal failure. Dents disease results from mutations of the voltage-gated chloride channel CLC-5. METHODS: We studied the effect of zero and high citrate diet on renal function of ClC-5 knockout mice and wild-type mice. The mice were placed in metabolic cages from which the urine was collected. Mice were sacrificed to obtain serum and tissues for analysis. RESULTS: ClC-5 knockout mice fed zero or high citrate diet had significantly increased urinary calcium excretion compared with wild-type mice fed the same diets. Nine-month-old ClC-5 knockout mice on a zero citrate diet had significantly decreased glomerular filtration rate (GFR), whereas 9-month-old ClC-5 knockout mice on a high citrate diet had normal renal function. ClC-5 knockout mice fed a zero citrate diet had significantly increased tubular atrophy, ...
TY - JOUR. T1 - Role of calcium in volume-activated chloride currents in a mouse cholangiocyte cell line. AU - Chen, Biyi. AU - Nicol, Grant. AU - Cho, Won Kyoo. PY - 2007/1/1. Y1 - 2007/1/1. N2 - Volume-activated Cl- channels (VACCs) play vital roles in many cells including cholangiocytes. Previously, we characterized the VACCs in mouse cholangiocytes. Since calcium plays an important role in VACC regulation in many cells, we have studied the effect of calcium modulation on the regulatory volume decrease (RVD) and VACC currents in mouse bile duct cells (MBDCs). Cell volume measurements were assessed by a Coulter counter with cell sizer, and conventional whole-cell patch-clamp techniques were used to study the role of calcium on RVD and VACC currents. Cell volume study indicated that MBDCs exhibited RVD, which was inhibited by 5-nitro-2′-(3-phenylpropylamino)- benzoate (NPPB), 4,4′-diisothiocyanostilbene-2,2′-disulfonate (DIDS) and 1,2-bis(o-aminophenoxy)ethane-N,N,N′,N′-tetraacetic ...
Abstract: : Purpose: In corneal endothelial cells, apical HCO3- permeability can be enhanced by increasing [Ca2+]i via either activation of purinergic receptor or inhibition of the sarcoendoplasmic reticulum calcium-ATPase ( SERCA ). Our goal is to identify the molecular candidate of the calcium-activated anion channel. Methods: Fresh and cultured bovine corneal endothelial cells ( FBCEC, CBCEC) were used in the study. A probe containing the first 457bp of the bCLCA1 open reading frame was used for in situ hybridization analysis. A 15 aa polypeptide (417-431) conjugate was used to generate polyclonal antibodies in rabbit. The immunoblot and immunofluoresence assay were performed to detect the protein expression of bCLCA1. Results: In situ hybridization with the bCLCA1-specific probe confirmed prominent mRNA expression in CBCEC. In immunoblot assay, the antiserum detects the heterologously expressed bCLCA1in HEK293 cell line, which was transfected with pcDNA3.1-bCLCA1 (a gift from Dr. Fuller in ...
Apical membrane chloride channels control chloride secretion by airway epithelial cells. Defective regulation of these channels is a prominent characteristic of cystic fibrosis. In normal intact cells, activation of protein kinase C (PKC) by phorbol ester either stimulated or inhibited chloride secretion, depending on the physiological status of the cell. In cell-free membrane patches, PKC also had a dual effect: at a high calcium concentration, PKC inactivated chloride channels; at a low calcium concentration, PKC activated chloride channels. In cystic fibrosis cells, PKC-dependent channel inactivation was normal, but activation was defective. Thus it appears that PKC phosphorylates and regulates two different sites on the channel or on an associated membrane protein, one of which is defective in cystic fibrosis. ...
TY - JOUR. T1 - Chloride equilibrium potential in salamander cones. AU - Thoreson, Wallace B. AU - Bryson, Eric J.. PY - 2004/12/5. Y1 - 2004/12/5. N2 - Background: GABAergic inhibition and effects of intracellular chloride ions on calcium channel activity have been proposed to regulate neurotransmission from photoreceptors. 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. Results: Whole cell recordings were done using gramicidin perforated patch techniques to maintain endogenous Cl- levels. Membrane potentials were corrected for liquid junction potentials. Cone resting potentials were found to average -46 mV. To measure ECl, we applied long depolarizing steps to activate the calcium-activated chloride current (ICl(Ca)) and then determined the reversal potential for the current component that was inhibited by the Cl- ...
This gene encodes a member of the bestrophin gene family. This small gene family is characterized by proteins with a highly conserved N-terminus with four to six transmembrane domains. Bestrophins may form chloride ion channels or may regulate voltage-gated L-type calcium-ion channels. Bestrophins are generally believed to form calcium-activated chloride-ion channels in epithelial cells but they have also been shown to be highly permeable to bicarbonate ion transport in retinal tissue. Mutations in this gene are responsible for juvenile-onset vitelliform macular dystrophy (VMD2), also known as Best macular dystrophy, in addition to adult-onset vitelliform macular dystrophy (AVMD) and other retinopathies. Alternative splicing results in multiple variants encoding distinct isoforms ...
TY - JOUR. T1 - Apical membrane chloride channels in a colonic cell line activated by secretory agonists. AU - Halm, D. R.. AU - Rechkemmer, G. R.. AU - Schoumacher, Robert. AU - Frizzell, R. A.. PY - 1988/1/1. Y1 - 1988/1/1. N2 - We characterized the anion channel responsible for the increase in apical membrane Cl secretion using a model salt-secreting epithelium, the T84 colonic cell line. The adenosine 3,5-cyclic monophosphate (cAMP)-mediated secretagogues, prostaglandin E2, forskolin, and 8-bromo-cAMP, evoked activity of an outwardly rectifying Cl channel in previously quiet cell-attached membrane patches. The channel remained active in excised, inside-out membranes, where its single-channel conductance was 40-45 pS at 0 mV with 160 mM NaCl in pipette and bath. Selectivities were P(Cl)/P(Na) = 50 and for halides I(1.8)/Br(1.4)/Cl(1.0)/F(0.4). This halide sequence illustrates that the ability of various anions to undergo transepithelial secretion is determined by the selectivity of the ...
SGLT1-based oral rehydration solution (ORS) used in the management of acute diarrhoea does not substantially reduce stool output, despite the fact that glucose stimulates the absorption of sodium and water. To explain this phenomenon, we investigated the possibility that glucose might also stimulate anion secretion. Transepithelial electrical measurements, isotope flux measurements in Ussing chambers, fluid movement in isolated ileal sacs and electrical measurements in patch clamp studies were used to study the effect of glucose on active chloride and fluid secretion in mouse small intestinal cells and human Caco-2 cells. Confocal fluorescence laser microscopy and immunohistochemistry measured intracellular changes in calcium, SGLT1 and calcium-activated chloride channel (anoctamin 1) expression. In addition to enhancing active sodium absorption, glucose increased intracellular calcium and stimulated electrogenic chloride secretion. Calcium imaging studies showed increased intracellular calcium ...
The data presented above indicate that Ano6, 8 and 9, when overexpressed in HEK293 cells, produce Cl− currents upon increase in intracellular Ca2+ by ionomycin. However, although all three paralogs are expressed endogenously in HEK293 cells, no currents are activated in mock-transfected cells (Fig. 3A) (Almaça et al., 2009). However, when increasing [Ca2+]i to very high levels (≥10 µM), endogenous anoctamins were activated (Fig. 3B,E). This may be due to a lack of sufficient membrane expression of endogenous anoctamins. Endogenous anoctamins are expressed at much lower levels than overexpressed proteins and may be inhibited by endogenous accessory proteins, such as CAMKII. Alternatively, specific Ca2+ release and/or influx pathways need to be triggered to activate these endogenous anoctamins. The overall linear structures appear similar in all anoctamins (supplementary material Fig. S1) and an analysis of putative functional domains revealed a number of similar motifs and putative ...
Tumor necrosis factor alpha inhibits signaling from the insulin receptor.. The medical assessment for police officer special constable police community support Diabetes. The Australasian Diabetes in Pregnancy Society consensus guidelines for the management of diabetes information websites breakfast meals type 1 and type 2 diabetes in relation to pregnancy Potassium Channels and Calcium-Activated Chloride Channels: and control of hormone release from endocrine tissues. Healthy diet and lifestyle choices form the basis of managing diabetes.. Q: Do mosquitoes prefer certain people over others? I could swear I always get bitten up more than my friends do when were together! Yes they do! canadian pharmacy insulin. If the blood sugar level Diabetes Journal Letter Tracker Test falls too low energy supply to all tissues particularly the ain is impaired. Wartenberg responded: Yes sometimes. What is insulin pump therapy? Insulin pumps use only et al. NICE - Endorsed Clinical Guidelines ...
Chloride channels (ClCs) have gained worldwide interest because of their molecular diversity, widespread distribution in mammalian tissues and organs, and their link to various human diseases. Nine different ClCs have been molecularly identified and functionally characterized in mammals. ClC-2 is one of nine mammalian members of the ClC family. It possesses unique biophysical characteristics, pharmacological properties, and molecular features that distinguish it from other ClC family members. ClC-2 has wide organ/tissue distribution and is ubiquitously expressed. Published studies consistently point to a high degree of conservation of ClC-2 function and regulation across various species from nematodes to humans over vast evolutionary time spans. ClC-2 has been intensively and extensively studied over the past two decades, leading to the accumulation of a plethora of information to advance our understanding of its pathophysiological functions; however, many controversies still exist. It is necessary to
Read "A Large-Conductance Chloride Channel in Pigmented Ciliary Epithelial Cells Activated by GTPγS, The Journal of Membrane Biology" on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips.
Introduction: Adult hearts contain c-Kit+ endogenous cardiac stem cells (eCSCs) that are being studied for regenerative therapy. eCSCs express volume-dependent Cl- channels (ICl,vol) activated under hemodynamic stress. Here, we examined the changes in eCSC Cl- channels caused by experimental congestive heart failure (CHF).. Methods: c-Kit+ eCSCs isolated from healthy (CTL) and CHF (tachypaced) dog hearts were magnetically purified with c-Kit antibodies. Cl- channel expression in freshly isolated cells was studied with patch clamp and qPCR.. Results: Under isotonic conditions, ionic currents were barely detectable in CTL eCSCs (Fig. A). The current observed was time-independent and reversed at values close to the calculated Cl- equilibrium potential (ECl-≈ -40 mV). This current was insensitive to TEA-Cl but inhibited by the Cl- channel blocker DIDS. When cells were superfused with hypo-osmotic solution (0.7T), an outward current with unchanged reversal potential was activated, and completely ...
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This chapter reviews the well-characterized genetic syndromes of bacterial susceptibility with an eye toward specificity of the susceptibility, applicability of the defect to infections in the general population, and, where applicable, treatment. Defects in barriers involving the skin and lung and defects of leukocyte number, trafficking, and function are also considered in this chapter. The major protection afforded by the skin is due to the stratum corneum, which provides a multifunctional barrier retarding water loss and providing an antimicrobial barrier. Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), the gene that encodes the major epithelial chloride channel. This chapter focuses on examples of innate immune defects, especially those involving phagocytic cells as mutations in the genes regulating the innate immune system have been very informative regarding the specific associations of genes and
Chloride channel accessory 1 is a protein that in humans is encoded by the CLCA1 gene. This gene encodes a member of the calcium sensitive chloride conductance protein family. To date, all members of this gene family map to the same region on chromosome 1p31-p22 and share a high degree of homology in size, sequence, and predicted structure, but differ significantly in their tissue distributions. The encoded protein is expressed as a precursor protein that is processed into two cell-surface-associated subunits, although the site at which the precursor is cleaved has not been precisely determined. The encoded protein may be involved in mediating calcium-activated chloride conductance in the intestine. Protein structure prediction methods suggest the N-terminal region of CLCA1 protein is a zinc metalloprotease. Chloride channel GRCh38: Ensembl release 89: ENSG00000016490 - Ensembl, May 2017 GRCm38: Ensembl release 89: ENSMUSG00000028255 - Ensembl, May 2017 "Human PubMed Reference:". "Mouse PubMed ...
A bicyclic fatty acid derived from prostaglandin E1 and a chloride channel activator with laxative activity. Upon intake, lubiprostone specifically binds to and activates the type 2 chloride channel (ClC-2) in the apical membrane of the gastrointestinal epithelium. This produces an efflux of chloride ions, thereby drawing water into the gastrointestinal lumen. The resulting increased amounts of intestinal fluid soften the stool, increase motility, and improve bowel movements.
We have shown that XE991 can reduce electrogenic chloride secretion in the murine colon when it is added basolaterally. This result supports the general thesis that hyperpolarization increases chloride efflux through the apical membrane by increasing the electrical gradient. Conversely, blockade of basolateral K+-channels, by causing depolarization, will reduce chloride secretion. Because XE991 reduces the basal SCC without prior stimulation of SCC, this suggests that some K+ channels are constitutively open, as has been argued by others (Schroeder et al., 2000).. Both forskolin and EBIO have multiple actions on chloride secretory epithelia. Both activate apically located CFTR chloride channels, either by increasing cAMP (Cuthbert et al., 1999b) or by direct action (Devor et al.,1996a,b), and both activate basolateral K+ channels, again either via cAMP or by a mixture of direct and indirect actions (Devor et al.,1996a,b; Cuthbert et al., 1999b; Syme et al., 2000). However, as shown here for the ...
CLC-5, the chloride channel mutated in Dents disease, colocalizes with the proton pump in endocytotically active kidney cells ...
This study reports the formation of self-assembled transmembrane anion channels by small-molecule fumaramides. Such artificial ion channel formation was confirmed by ion transport across liposomes and by planar bilayer conductance measurements. The geometry-optimized model of the channel and Cl− ion selectivity wit
Membrane Protein Disease, Innate Immunity. Plasma membrane (PM) proteins including signaling receptors, ion channels and transporters play crucial roles in physiological functions of cells and organisms. Genetic and environmental stresses cause the defective expression of PM proteins that is associated with several human diseases such as cystic fibrosis (CF). The ultimate goal in our research is to elucidate the molecular and cellular mechanisms underlying the defective expression of PM proteins and to help develop novel therapeutic approaches for human diseases.. Right now, we focus on the CFTR chloride channel associated with CF, one of the most common genetic diseases in Caucasians. While the most common CFTR mutant in CF patients has the ability to function as a chloride channel, it is rapidly eliminated by proteolysis through cellular quality control systems including the PM quality control mechanism that we originally identified. We are interested in understanding the molecular and ...
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A high-affinity specific binding site is reported for [3H]AVM B1a with K Dvalues of 1.4 nM as a rough estimate for dog brain (Pong and Wang, 1980) and 2.5 nM for rat cerebellar cortex (Drexler and Sieghart, 1984a) and for its dihydro analog [3H]ivermectin with aK D value of 22 nM for rat cerebral cortex (Schaeffer and Haines, 1989). In our preliminary study, we found that the specific binding sites for AVM B1a are widely distributed in rat whole brain but particularly enriched in the cerebellum, which is consistent with the findings of Pong and Wang (1980). We then evaluated the binding properties of [3H]AVM B1a in primary cultures of cerebellar neurons and directly investigated the effects of AVM B1a on the GABA-gated chloride channel with [3H]EBOB binding and36Cl− influx as indicators. The use of intact neuron cultures made it possible to perform radioligand binding and functional assays of 36Cl−influx in comparable and physiological conditions.. Specific binding of [3H]AVM B1a in intact ...
Moral-Sanz, J., Mahmoud, A. D., Ross, F. A., Eldstrom, J., Fedida, D., Hardie, D. G. & Evans, A. M. 1 Sep 2016 In : Journal of Physiology. 594, 17, p. 4901-4915 15 p.. Research output: Contribution to journal › Article ...
Zhang CH, Wang P, Liu DH, Chen CP, Zhao W, Chen X, Chen C, He WQ, Qiao YN, Tao T, Sun J, Peng YJ, Lu P, Zheng K, Craige SM, Lifshitz LM, Keaney JF, Fogarty KE, ZhuGe R, Zhu MS. The molecular basis of the genesis of basal tone in internal anal sphincter. Nat Commun. 2016 Apr 22; 7:11358 ...
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Synonyms for acetyl chloride in Free Thesaurus. Antonyms for acetyl chloride. 1 synonym for acetyl chloride: ethanoyl chloride. What are synonyms for acetyl chloride?
This gene encodes a serine/threonine protein kinase that plays an important role in cellular stress response. This kinase activates certain potassium, sodium, and chloride channels, suggesting an involvement in the regulation of processes such as cell survival, neuronal excitability, and renal sodium excretion. High levels of expression of this gene may contribute to conditions such as hypertension and diabetic nephropathy. Several alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jan 2009 ...
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BEST1 Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 317 aa (292-585) having a molecular mass of 36 kDa.
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This test will find out how much chloride is in your blood and help your healthcare provider find out if you have a kidney problem.
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Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis Academic Article ...
The present invention relates to chloride-selective electrodes which comprise insoluble metal salt layer and a protecting membrane formed of hydrophilic polyurethane thereon, wherein the hydrophilic polyurethane coated chloride-selective electrodes show fast activation and response time and are usefully employed to accurately measure the chloride by reducing the interference from bromide and iodide or preventing the surface of the electrode from protein adsorption.
Ca2+-activated Cl- channels (CaCCs) participate in many important physiological processes. However, the lack of effective and selective blockers has hindered the study of these channels, mostly due to the lack of good assay system. Here, we have developed a reliable drug screening method for better blockers of CaCCs, using the endogeneous CaCCs in Xenopus laevis oocytes and two-electrode voltage-clamp (TEVC) technique. Oocytes were prepared with a treatment of Ca2+ ionophore, which was followed by a treatment of thapsigargin which depletes Ca2+ stores to eliminate any contribution of Ca2+ release. TEVC was performed with micropipette containing chelerythrine to prevent PKC dependent run-up or run-down. Under these conditions, Ca2+-activated Cl- currents induced by bath application of Ca2+ to oocytes showed stable peak amplitude when repetitively activated, allowing us to test several concentrations of a test compound from one oocyte. Inhibitory activities of commercially available blockers and
The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen). Alternative splicing results in multiple transcript variants ...
Cystic fibrosis (CF) is caused by loss-of-function mutations in the CF transmembrane conductance regulator (CFTR) Cl- channel. We developed a phenotype-based high-throughput screen to identify small-molecule activators of human airway epithelial Ca2+-activated Cl- channels (CaCCs) for CF therapy. Unexpectedly, screening of ~110,000 synthetic small molecules revealed an amino-carbonitrile-pyrazole, Cact-A1, that activated CFTR but not CaCC Cl- conductance. Cact-A1 produced large and sustained CFTR Cl- currents in CFTR-expressing Fisher rat thyroid (FRT) cells and in primary cultures of human bronchial epithelial (HBE) cells, without increasing intracellular cAMP and in the absence of a cAMP agonist. Cact-A1 produced linear whole-cell currents. Cact-A1 also activated ΔF508-CFTR Cl- currents in low temperature-rescued ΔF508-CFTR-expressing FRT cells and CF-HBE cells (from homozygous ΔF508 patients) in the absence of a cAMP agonist, and showed additive effects with forskolin. In contrast, VX-770 ...
Fig. 58 shows the progression of adult vitelliform macular dystrophy (AVMD) in a 50-year-old female. Images are a series of ocular coherence tomograhic pictures. During the initial stage, similar to the first OCT dated August 25, 2011 is when the ocular fundus may have "sunny-side-up" egg yolk appearance. In later stages the lipofuscin disperses resulting in "scrambled egg" appearance with mottled pigmentation and RPE atrophy.. Fig. 58 shows the progression of adult vitelliform macular dystrophy (AVMD) in a 50-year-old female. During the initial stage similar to the first OCT dated August 25, 2011, is when the ocular fundus may have a "sunny-side-up" egg yolk appearance. In later stages the lipofuscin disperses resulting in a "scrambled egg" appearance with mottled pigmentation and RPE atrophy. In Bests disease and in some with AVMD, a dysfunction of bestrophin results in abnormal fluid and ion transport by the retinal pigment epithelium (RPE). Bestrophins are a family of proteins that can ...
Modulation of gamma-aminobutyric acidA receptor-operated chloride channels by benzodiazepine inverse agonists is related to genetic differences in ethanol withdrawal seizure severity. Article date: 1991/12/1 PubMed ID: 1658234 Journal name: Journal of neurochemistry (ISSN: 0022-3042)
Purpose : Primary open-angle glaucoma (POAG), Alzheimers diseases (AD), and adult macular degeneration (AMD) are common neurodegenerative diseases. Beta-amyloid (βA) deposition is widely believed to be a key pathological event of these diseases. Our laboratory has identified microvascular abnormalities and superactivated platelets (SAPs) in these diseases. The phosphatidylserine (PS) flip in which the inside of the plasma membrane everts to the outside is required for SAPs formation and occurs in blood coagulation, rod outer segment shedding, and removal of apoptotic cells. Methods : Using a proteomic approach, a calcium activated chloride channel, TMEM16F, was identified as candidate "flippase." Whole blood was obtained from controls after informed consent approved by the Univ. Il. at Chicago IRB, anticoagulated, centrifuged at 200xg for 15 min to isolate plasma rich platelets (PRP), washed with Tyrodes buffer and resuspended in FACS buffer. PRP (1 × 10-8/mL) was challenged with the calcium ...
Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. A cycle of chronic, persistent infections with CF-related pathogens and an excessive inflammatory response progressively damages the airways and lung parenchyma, resulting in widespread bronchiectasis and ultimately, respiratory failure. Despite tremendous advances in understanding the CF gene and the CFTR protein, it is not known exactly how mutations in the gene and defects in CFTR lead to persistent airway infection and inflammation.. Inhaled nitric oxide (NO) has potential to be an effective treatment in CF lung disease. Inhaled NO has been studied in other airways diseases characterized by infection and /or inflammation such as COPD and idiopathic pulmonary fibrosis.. NO has been shown to activate CFTR and alternative chloride channels, thereby increasing chloride current in epithelial cells. Therefore, NO treatment may be ...
1CKY: Cystic fibrosis transmembrane conductance regulator: solution structures of peptides based on the Phe508 region, the most common site of disease-causing DeltaF508 mutation.
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The cognitive enhancer XE991 interacts with K(+) channels consisting of KCNQ2 and KCNQ3 heteromultimers to block the M-current. XE991 can also block KCNQ1 K(+) channels expressed in oocytes, but sensitivity is reduced when the channels are coexpressed with minK (KCNE1). The purpose of the study was to examine the interaction of XE991 with other types of K(+) channel, especially those in the basolateral membranes of murine epithelia. K(+) channel blockade was measured by the inhibition of chloride secretion resulting from depolarization. XE991 inhibited the chloride secretory current in colonic epithelia by an interaction with basolateral K(+) channels when forskolin was used as the stimulus. However, when 1-ethyl-2-benzimidazolinone (EBIO) was used to stimulate chloride secretion, XE991 was ineffective unless charybdotoxin was also present. Because EBIO also activates Ca(2+)-sensitive K(+) channels, whereas forskolin activates only cAMP-sensitive K(+) channels, it is concluded that the latter ...
Membrane, Mutations, Cystic Fibrosis, Fibrosis, Mutation, ATP, Endoplasmic Reticulum, Reticulum, Chloride Channel, Disease, Cystic Fibrosis Transmembrane Conductance Regulator, Cells, Retention, Atpase, Cftr Protein, Epithelial Cells, Phosphorylation, Proteins, Role, Cell
Chronic cerebral hypoperfusion is believed to cause white matter lesions (WMLs), leading to cognitive impairment. Previous studies have shown that inflammation and apoptosis of oligodendrocytes (OLs) are involved in the pathogenesis of WMLs, but effective treatments have not been studied. In this study, 4,4-diisothiocyanostilbene-2,2-disulfonic acid (DIDS), a chloride (Cl−) channel blocker, was injected into chronic cerebral ischemia-hypoxia rat models at different time points. Our results showed that DIDS significantly reduced the elevated mRNA levels and protein expression of chloride channel 2 (ClC-2) in neonatal rats induced by ischemia-hypoxia. Meanwhile, DIDS application significantly decreased the concentrations of reactive oxygen species (ROS); and the mRNA levels of inducible nitric oxide synthase (iNOS) and tumor necrosis factor-alpha TNF-α in neonatal rats with hypoxic-ischemic damage. Myelin staining was weaker in neonatal rats with hypoxic-ischemic damage compared to normal controls
Summary of Facts and Submissions. I. The appellant (applicant) lodged an appeal against the decision of the examining division on the refusal under Article 97(1) EPC 1973 of the European patent application No. 01 964 677.7 (published as WO-A-01/74884), having the title Dermacentor variabilis GABA-gated chloride channels.. II. In its decision for refusing the present application, the examining division referred to the reasons given in its communication dated 29 August 2007, wherein it raised an objection on the ground of lack of inventive step only.. III. The decision under appeal was based on claims 1 and 2 filed with the applicants letter dated 08 August 2007, which is now the main request before the board, reading as follows:. 1. A purified polypeptide, wherein said polypeptide comprises an amino acid sequence selected from SEQ. ID. NO. 1, SEQ. ID. NO. 2, and SEQ. ID. NO. 3.. 2. The polypeptide of claim 1, wherein said polypeptide consists of an amino acid sequence selected from SEQ. ID. ...
Cystic fibrosis (CF) is an autosomal recessive disease that greatly diminishes life span owing to impaired function of the lungs and intestinal epithelia. Although the F508 homozygous mutation in the anion channel protein cystic fibrosis transmembrane conductance regulator (CFTR) is commonly found in CF patients, the mechanisms by which this mutation causes disease are unclear. To investigate this issue in vivo, Ostedgaard et al. generated pigs homozygous for CFTR-F508. Unlike previously established mouse models of CF, CFTR-F508 pigs develop airway and intestinal disease that closely resembles human CF pathology. In vivo studies agreed with previous results carried out in cell culture systems, and showed that the F508 mutation causes CFTR misfolding and degradation, and the failure of the protein to localise at the apical surface of epithelial cells. CFTR-F508 pigs provide a relevant model to further investigate mechanisms of CF pathogenesis and for testing therapies that aim to increase CFTR ...
Volumenregulation ist ein grundlegender Mechanismus, der für alle lebenden Organismen notwendig ist. Zellen schwellen unter einem hypo- osmotischen Schock und aktivieren darauf die Freisetzung von K+ und Cl- Ionen über die Aktivierung von K+- und Cl-- Kanälen, um das Ausgangsvolumen wieder herzustellen. Im Jahre 2014 wurde von zwei unabhängigen Arbeitsgruppen LRRC8A als Hauptkomponente des Volumen- Regulierten Anionen Canals (VRAC) identifiziert. Die Ergebnisse der vorliegenden Arbeit lassen aber vermuten, dass auch Anoctamine und Bestrophine an der Volumenregulation beteiligt sind. Darüber hinaus konnte für diese Proteine der Mechanismus ihrer zellvolumen-abhängigen Aktivierung geklärt werden. Zusätzlich wurde in dieser Arbeit die Rolle der TMC Proteine (TransMembrane Channel-like family of Proteins), die eine hohe Sequenzähnlichkeit zu den Anoctaminen besitzen, für die Volumenregulation und für die Regulation der intrazellulären Ca2+ Konzentration untersucht. Überexpression von ...
囊狀纖維化(CF)是一種遺傳性的基因突變疾病,屬於體染色體隱性遺傳,在白種人較為常見,帶因比例約為3%,發病率約為1/3300,而其中亞裔僅佔1/32000。近年來,隨著國人與外籍人士通婚比例增加,可能提高此遺傳疾病之發生率,但目前仍然屬於國內十分罕見的疾病之一。. 囊狀纖維化的基因突變位在第七對染色體長臂 7q31.2 區域的鹼基對上,進而導致人體中特定蛋白功能的缺陷,這些蛋白被稱為「囊狀纖維化跨膜傳導調節節因子」(CFTR, cystic fibrosis transmembrane conductance ...
Looking for online definition of cystic fibrosis transmembrane conductance regulator gene in the Medical Dictionary? cystic fibrosis transmembrane conductance regulator gene explanation free. What is cystic fibrosis transmembrane conductance regulator gene? Meaning of cystic fibrosis transmembrane conductance regulator gene medical term. What does cystic fibrosis transmembrane conductance regulator gene mean?
Cystic fibrosis transmembrane conductance regulator (CFTR) has been considered to be involved in the regulatory pathway of biliary mucin secretion. We investigated expression of CFTR protein and mRNA in 24 livers with hepatolithiasis, in 6 with cholangiocarcinoma, and in 12 histologically normal livers. According to the histologic features of chronic proliferative cholangitis, hepatolithiasis was subdivided into inflammatory cell infiltration predominant (N = 14) and fibrosis predominant (N = 10). The mean signal density of CFTR in overall hepatolithiasis and in histologically normal livers was 1.23 ± 0.15 and 1.01 ± 0.13, respectively (P | 0.05). The CFTR protein (1.60 ± 0.18) and mRNA (1.09 ± 0.15) in inflammatory cell infiltration predominant patients were significantly higher (CFTR protein, 1.01 ± 0.13; mRNA, 0.75 ± 0.11) than in control subjects (P | 0.05), whereas those in fibrosis-predominant patients (CFTR protein, 0.72 ± 0.15; mRNA, 0.55 ± 0.13) were less than in control subjects (P | 0
TY - JOUR. T1 - Superoxide anion radicals activate hepatic stellate cells after entry through chloride channels. T2 - A new target in liver fibrosis. AU - den Hartog, G.J.. AU - Qi, S.. AU - van Tilburg, J.H.. AU - Koek, G.H.. AU - Bast, A.. PY - 2014/2/5. Y1 - 2014/2/5. N2 - It is generally accepted that reactive oxygen species (ROS) play an important role in the pathogenesis of liver fibrosis. ROS, however, constitute a group of species with varying properties making it likely that their contribution to the pathological mechanism varies. LX-2 hepatic stellate cells (HSCs) were exposed to superoxide anion radicals (O2−) generated by xanthine and xanthine oxidase. To rule out that the activation of HSCs is due to hydrogen peroxide derived from O2−, control incubations with copper, zinc-superoxide dismutase and tempol were studied as well. Influx of O2− activated HSCs, evidenced by the expression of α-smooth muscle actin and the secretion of transforming growth factor β1 and collagen. We ...
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The chromosomal localization of the gene for Thomsen disease, an autosomal dominant form of myotonia congenita, is unknown. Electrophysiologic data in Thomsen disease point to defects in muscle-membrane ion-channel function. A mouse model of myotonia congenita appears to result from transposon inactivation of a muscle chloride-channel gene which maps to a region of mouse chromosome 6. The linkage group containing this gene includes several loci which have human homologues on human chromosome 7q31-35 (synteny), and this is a candidate region for the Thomsen disease locus. Linkage analysis of Thomsen disease to the T-cell-receptor beta (TCRB) locus at 7q35 was carried out in four pedigrees (25 affected and 23 unaffected individuals) by using a PCR-based dinucleotide repeat polymorphism in the TCRB gene. Two-point linkage analysis between Thomsen disease and TCRB showed a maximum cumulative lod score of 3.963 at a recombination fraction of .10 (1-lod support interval .048-.275). We conclude that the
Purpose: Objective pupilloperimetry in healthy subjects and patients with Bests Vitelliform Macular Dystrophy using a chromatic multifocal pupillometer (CMP). Methods: A CMP (Accutome Inc) was used to record pupillary responses (PR) of 17 healthy subjects and 5 Bests patients. Red and blue light stimuli were presented at 76 locations of a 16.2 degree VF. The PR of patients were compared with their findings on Humphreys 24-2 perimetry, Optical Coherence Tomography (OCT) and with the PR of healthy subjects. Percentage of Pupil Constriction (PPC), maximal constriction velocity (MCV) and the latency of MCV (LMCV) were determined. Results: In response to red light, Bests patients demonstrated reduced PPC and slower MCV compared with healthy subjects in nearly all test point locations. Severity of defects in PPC in responses to red light correlated with reduced thickness of photoreceptor layer as determined by OCT in the central, superior, nasal and temporal areas of the central retina (Pearsons ...
NIH Rare Diseases : 50 best vitelliform macular dystrophy (bvmd) is a slowly progressive form of macular degeneration. it usually begins in childhood or adolescence, but age of onset and severity of vision loss can vary. affected people first have normal vision, followed by decreased central visual acuity and distorted vision (metamorphopsia). peripheral vision is not affected. bvmd is characterized by atrophy of the retinal pigment epithelium (the retina is the back part of the eye that contains the specialized cells that respond to light, known as photoreceptors) and impaired central visual function. bvmd is usually inherited in an autosomal dominant manner, but autosomal recessive inheritance has been reported. the condition is typically caused by mutations in the best1 gene; in a few cases the cause is unknown. treatment is symptomatic and involves the use of low vision aids, and direct laser treatment or photodynamic therapy. newer treatment includes anti-vegf agents (bevacizumab) and ...

CLIC4 (chloride intracellular channel 4)CLIC4 (chloride intracellular channel 4)

... chloride intracellular channel 4), Authors: Velayuthan C Padmakumar, Stuart H Yuspa. Published in: Atlas Genet Cytogenet Oncol ... angiogenesis endothelial cell morphogenesis voltage-gated ion channel activity chloride channel activity protein binding ... angiogenesis endothelial cell morphogenesis voltage-gated ion channel activity chloride channel activity protein binding ... CLIC4 (chloride intracellular channel 4). Written. 2009-07. Velayuthan C Padmakumar, Stuart H Yuspa. ...
more infohttp://atlasgeneticsoncology.org/Genes/CLIC4ID40102ch1p36.html

Chloride intracellular channel 1 participates in migration and invasion of hepatocellular carcinoma by targeting maspinChloride intracellular channel 1 participates in migration and invasion of hepatocellular carcinoma by targeting maspin

Our previous proteomic research found that chloride intracellular channel 1 (CLIC1) was upregulated in hepatocellular carcinoma ... Chloride intracellular channel 1 participates in migration and invasion of hepatocellular carcinoma by targeting maspin ... Background and Aim:Our previous proteomic research found that chloride intracellular channel 1 (CLIC1) was upregulated in ...
more infohttps://insights.ovid.com/jgahe/201501000/00001753-201501000-00034

Macromolecular conjugates of cystic fibrosis transmembrane conductance     regulator protein inhibitors and uses therefor -...Macromolecular conjugates of cystic fibrosis transmembrane conductance regulator protein inhibitors and uses therefor -...

Yang et al., "Nanomolar Affinity Small Molecule Correctors of Defective .DELTA.F508-CFTR Chloride Channel Gating" The Journal ... Schultz et al., "Pharmacology of CFTR Chloride Channel Activity" Physiological Reviews 79(Supp. No. 1): S109-S144, 1999. cited ... Verkman et al., "CFTR Chloride Channel Drug Discovery--Inhibitors as Antidiarrheals and Activators for Therapy of Cystic ... McCarty, "Permeation Through the CFTR Chloride Channel" The Journal of Experimental Biology 203: 1947-1962, 2000. cited by ...
more infohttp://www.patentgenius.com/patent/8552067.html

Cystic fibrosis transmembrane conductance regulator: a molecular model defines the architecture of the anion conduction path...Cystic fibrosis transmembrane conductance regulator: a molecular model defines the architecture of the anion conduction path...

... consistent with the observation that cysteines at both of these locations reacted with both channel-permeant and channel- ... were found to react exclusively with channel-permeant reagents applied from the extracellular side. The predicted dimensions of ... We developed molecular models for the cystic fibrosis transmembrane conductance regulator chloride channel based on the ... We developed molecular models for the cystic fibrosis transmembrane conductance regulator chloride channel based on the ...
more infohttps://www.neuroscience.ox.ac.uk/publications/322751

Chloride channel - WikipediaChloride channel - Wikipedia

Chloride channels are a superfamily of poorly understood ion channels specific for chloride. These channels may conduct many ... The chloride channel subunits contain between 1 and 12 transmembrane segments. Some chloride channels are activated only by ... Based on sequence homology the chloride channels can be subdivided into a number of groups. Voltage-gated chloride channels are ... CLC chloride channels As of this edit, this article uses content from "1.A.13 The Epithelial Chloride Channel (E-ClC) Family", ...
more infohttps://en.wikipedia.org/wiki/Chloride_channel

Regulation of Lymphocyte Chloride Channels | SpringerLinkRegulation of Lymphocyte Chloride Channels | SpringerLink

The chloride permeability defect which characterizes the apical membrane of secretory epithelial cells in cystic fibrosis has ... This channel is closely similar or identical to the outwardly rectifying chloride channel identified by patch clamp recording ... Cystic Fibrosis Chloride Channel Calcium Ionophore Patch Clamp Recording Secretory Epithelial Cell These keywords were added by ... Welsh, M.J., 1986, An apical-membrane chloride channel in human tracheal epithelium, Science, 23 2:1648.CrossRefGoogle Scholar ...
more infohttps://link.springer.com/chapter/10.1007/978-1-4684-5934-0_30

Dimeric structure of single chloride channels from Torpedo electroplax | PNASDimeric structure of single chloride channels from Torpedo electroplax | PNAS

Dimeric structure of single chloride channels from Torpedo electroplax Message Subject (Your Name) has sent you a message from ... Dimeric structure of single chloride channels from Torpedo electroplax. C Miller and M M White ... At the single-channel level, the effect of DIDS is more complicated. The uninhibited single channel displays three "substates" ... Short exposure (5-30 s) to 10 microM DIDS converts this three-level active channel into a "conventional" channel of 10-pS ...
more infohttps://www.pnas.org/content/81/9/2772?ijkey=d34950de896eec91a1ccdb23bcf8459fb1fa5f23&keytype2=tf_ipsecsha

Chloride channels: a molecular perspective | MDC BerlinChloride channels: a molecular perspective | MDC Berlin

Structurally, three classes of Cl- channels have been identified: ligand-gated, postsynaptic Cl- channels (e.g. GABA and ... channel and its emergent role as a regulator of other channels, clarification of issues related to swelling-activated Cl- ... channels. Recent developments of note include further characterization of the expanding CLC Cl- channel family, advances in ... Plasma membrane Cl- channels perform a variety of functions, including control of excitability in neurons and muscle, cell ...
more infohttps://www.mdc-berlin.de/research/publications/chloride-channels-molecular-perspective

chloride channel 6 ELISA Kits | Biocompare.comchloride channel 6 ELISA Kits | Biocompare.com

Compare chloride channel 6 ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, ... Bovine Chloride intracellular channel protein 6 (CLIC6) ELISA Kit MyBioSource.com *Detection Target: Chloride intracellular ... Canine Chloride intracellular channel protein 6 (CLIC6) ELISA Kit MyBioSource.com *Detection Target: Chloride intracellular ... Goat Chloride intracellular channel protein 6 (CLIC6) ELISA Kit MyBioSource.com *Detection Target: Chloride intracellular ...
more infohttps://www.biocompare.com/pfu/110627/soids/2-317936/Assay_Kit/ELISA_chloride_channel_6?vcmpv=true

chloride channel 6 ELISA Kits | Biocompare.comchloride channel 6 ELISA Kits | Biocompare.com

Compare chloride channel 6 ELISA Kits from leading suppliers on Biocompare. View specifications, prices, citations, reviews, ... chloride channel 6 ELISA Kits. The ELISA (enzyme-linked immunosorbent assay) is a well-established antibody-based tool for ... Your search returned 23 chloride channel 6 ELISA ELISA Kit across 1 supplier. ...
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Chloride channels - Biology-Online Dictionary | Biology-Online DictionaryChloride channels - Biology-Online Dictionary | Biology-Online Dictionary

Retrieved from "https://www.biology-online.org/dictionary/index.php?title=Chloride_channels&oldid=14859" ...
more infohttps://www.biology-online.org/dictionary/Chloride_channels

Gating the Selectivity Filter in ClC Chloride Channels | ScienceGating the Selectivity Filter in ClC Chloride Channels | Science

ClC channels conduct chloride (Cl-) ions across cell membranes and thereby govern the electrical activity of muscle cells and ... The structural basis of ClC channel gating was studied. Crystal structures of wild-type and mutant Escherichia coliClC channels ... Mutations of this glutamate residue in Torpedo ray ClC channels alter gating in electrophysiological assays. These findings ...
more infohttps://science.sciencemag.org/content/300/5616/108.long

WikiGenes - Clcn5 - chloride channel 5WikiGenes - Clcn5 - chloride channel 5

Synonyms: 5430408K11Rik, Chloride channel protein 5, Chloride transporter ClC-5, ClC-5, Clc5, ... ... The loss of the chloride channel, ClC-5, delays apical iodide efflux and induces a euthyroid goiter in the mouse thyroid gland. ... Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules. ... The ClC-5 chloride channel knock-out mouse - an animal model for Dents disease. Günther, W., Piwon, N., Jentsch, T.J. Pflugers ...
more infohttps://www.wikigenes.org/e/gene/e/12728.html

Chloride Channels | Profiles RNSChloride Channels | Profiles RNS

"Chloride Channels" by people in this website by year, and whether "Chloride Channels" was a major or minor topic of these ... "Chloride Channels" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical Subject ... A tyramine-gated chloride channel coordinates distinct motor programs of a Caenorhabditis elegans escape response. Neuron. 2009 ... Cell membrane glycoproteins that form channels to selectively pass chloride ions. Nonselective blockers include FENAMATES; ...
more infohttps://profiles.umassmed.edu/display/124078

chloride channel activity QuickView - Correlation Enginechloride channel activity QuickView - Correlation Engine

... involving passage through a transmembrane aqueous pore or channel without evidence for a carrier-mediated mechanism. ... Catalysis of facilitated diffusion of a chloride (by an energy-independent process) ... TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity. * Alpha-aminoazaheterocyclic- ... Extracellular recording of glycine receptor chloride channel activity as a prototype for biohybrid s… ...
more infohttp://www.nextbio.com/b/search/ov/chloride%20channel%20activity?id=33680&type=biogroup

Calcium-activated chloride channel regulator 1 precursor - Bos taurus (Bovine)Calcium-activated chloride channel regulator 1 precursor - Bos taurus (Bovine)

May be involved in mediating calcium-activated chloride conductance (PubMed:8537359). May play critical roles in goblet cell ... Calcium-activated chloride channel. Epithelial chloride channel protein1 Publication. ,p>Manually curated information that is ... Calcium-activated chloride channel regulator 1Add BLAST. 882. Amino acid modifications. Feature key. Position(s). Description ... Calcium-activated chloride channel regulator 1Curated (EC:3.4.-.-By similarity. ,p>Manually curated information which has been ...
more infohttp://www.uniprot.org/uniprot/P54281

The ABC protein turned chloride channel whose failure causes cystic fibrosis.  - PubMed - NCBIThe ABC protein turned chloride channel whose failure causes cystic fibrosis. - PubMed - NCBI

CFTR chloride channels are encoded by the gene mutated in patients with cystic fibrosis. These channels belong to the ... The ABC protein turned chloride channel whose failure causes cystic fibrosis.. Gadsby DC1, Vergani P, Csanády L. ... But channels do not stop opening until ATP is removed; numbers of simultaneously open channels are indicated at the right. b, ... The recording shows chloride current flow through individual CFTR channels upon opening. Endogenous membrane-attached ...
more infohttps://www.ncbi.nlm.nih.gov/pubmed/16554808?dopt=Abstract

WikiGenes - CLCC1 - chloride channel CLIC-like 1WikiGenes - CLCC1 - chloride channel CLIC-like 1

Synonyms: Chloride channel CLIC-like protein 1, KIAA0761, MCLC, Mid-1-related chloride channel protein 1 ...
more infohttps://www.wikigenes.org/e/gene/e/23155.html

Studies on expression and function of the TMEM16A calcium-activated chloride channel | PNASStudies on expression and function of the TMEM16A calcium-activated chloride channel | PNAS

Variomics Screen Identifies the Re-entrant Loop of the Calcium-activated Chloride Channel ANO1 That Facilitates Channel ... 1992) Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia. Am J Physiol 263: ... Calcium-activated chloride channels have been implicated for the STIC and also the plateau phase of depolarization in the slow ... Studies on expression and function of the TMEM16A calcium-activated chloride channel. Fen Huang, Jason R. Rock, Brian D. Harfe ...
more infohttps://www.pnas.org/content/106/50/21413?ijkey=79d83299dab6c8026f281254449d21addf393000&keytype2=tf_ipsecsha

anti-Chloride Channel, Nucleotide-Sensitive, 1A Primary Antibodiesanti-Chloride Channel, Nucleotide-Sensitive, 1A Primary Antibodies

methylosome subunit pICln , chloride channel, nucleotide-sensitive, 1A , chloride channel regulatory protein , chloride channel ... chloride channel current inducer , chloride channel regulator , Clns 1a , swelling-induced chloride conductance regulatory ... Weitere Produktkategorien zu Chloride Channel, Nucleotide-Sensitive, 1A Antikörper * 59 anti-Chloride Channel, Nucleotide- ... Weitere Antikörper gegen Chloride Channel, Nucleotide-Sensitive, 1A Interaktionspartner. Human Chloride Channel, Nucleotide- ...
more infohttps://www.antikoerper-online.de/ribonucleoprotein-complex-subunit-organization-pathway-73/clns1a-antibody-1255/

Clca1 MGI Mouse Gene Detail - MGI:1346342 - chloride channel accessory 1Clca1 MGI Mouse Gene Detail - MGI:1346342 - chloride channel accessory 1

IPR013642 Calcium-activated chloride channel, N-terminal. IPR004727 Calcium-activated chloride channel protein, chordata ...
more infohttp://www.informatics.jax.org/marker/MGI:1346342

Gating the Selectivity Filter in ClC Chloride Channels | ScienceGating the Selectivity Filter in ClC Chloride Channels | Science

ClC channels conduct chloride (Cl-) ions across cell membranes and thereby govern the electrical activity of muscle cells and ... The structural basis of ClC channel gating was studied. Crystal structures of wild-type and mutant Escherichia coliClC channels ... Mutations of this glutamate residue in Torpedo ray ClC channels alter gating in electrophysiological assays. These findings ...
more infohttp://science.sciencemag.org/content/300/5616/108

Clcn1 - Chloride channel protein 1 - Mus musculus (Mouse) - Clcn1 gene & proteinClcn1 - Chloride channel protein 1 - Mus musculus (Mouse) - Clcn1 gene & protein

IPR014743 Cl-channel_core. IPR001807 Cl-channel_volt-gated. IPR002243 Cl_channel-1. ... Chloride channel protein 1Imported. ,p>Information which has been imported from another database using automatic procedures.,/p ... tr,F6QL23,F6QL23_MOUSE Chloride channel protein 1 (Fragment) OS=Mus musculus OX=10090 GN=Clcn1 PE=4 SV=1 ... Chloride channel protein 1. Chloride channel protein 1, ClC-1 (Chloride channel protein, skeletal muscle) ...
more infohttps://www.uniprot.org/uniprot/F6QL23.html

Chloride channel-mediated brain glioma targeting of chlorotoxin-modified doxorubicine-loaded liposomes.  - PubMed - NCBIChloride channel-mediated brain glioma targeting of chlorotoxin-modified doxorubicine-loaded liposomes. - PubMed - NCBI

Chloride channel-mediated brain glioma targeting of chlorotoxin-modified doxorubicine-loaded liposomes.. Xiang Y, Liang L, Wang ...
more infohttps://www.ncbi.nlm.nih.gov/pubmed/21435361

Gene mutation in the chloride channel triggers rare high blood pressure syndromeGene mutation in the chloride channel triggers rare high blood pressure syndrome

MDC »Molekulare Pharmakologie »aldosterone »blood pressure »chloride »chloride channel »high blood pressure »mouse model » ... Further reports about: , MDC , Molekulare Pharmakologie , aldosterone , blood pressure , chloride , chloride channel , high ... Chloride channel continuously open. "We have seen how the channel is constantly open due to these mutations, which greatly ... "We have been able to show step by step what exactly happens in the organism due to the mutated chloride channel in our model ...
more infohttps://www.innovations-report.com/html/reports/life-sciences/gene-mutation-in-the-chloride-channel-triggers-rare-high-blood-pressure-syndrome.html
  • This problem has fostered attempts by investigators both to immortalize epithelial cell lines and to search for other cells which may serve as a model to study chloride conductances. (springer.com)
  • A genetic human disease, myotonia congenita, helped to clarify the impact of muscle chloride channels and provided an example of how background conductances can contribute to the modulation of cell excitability. (rupress.org)
  • and are perfectly suited to generate large resting chloride conductances while minimally interfering with the upstroke of the action potential. (rupress.org)
  • Voltage-gated chloride channels display a variety of important physiological and cellular roles that include regulation of pH, volume homeostasis, organic solute transport, cell migration, cell proliferation and differentiation. (wikipedia.org)
  • The TMEM16A expression pattern established in this study thus provides a roadmap for the analyses of physiological functions of calcium-activated chloride channels that contain TMEM16A subunits. (pnas.org)
  • Unlike glutamate, selamectin activates the chloride current without desensitization, thereby producing prolonged hyperpolarization and impaired muscle contraction. (bionity.com)
  • We have been able to show step by step what exactly happens in the organism due to the mutated chloride channel in our model for the first time," said the biologist. (innovations-report.com)
  • In a landmark study on muscle excitability and myotonia, Adrian and Bryant (1974) demonstrated that the existence of transverse tubules, deep invaginations of the sarcolemma that permit the quick penetration of electrical signals to the muscle interior, makes muscle chloride channels necessary for normal muscle excitability. (rupress.org)
  • Dysfunction of muscle chloride channels in myotonia does not modify the shape of the action potential ( Lipicky and Bryant, 1973 ), and so it can be concluded that these channels do not contribute to the repolarization of the muscle fiber. (rupress.org)
  • Heterologous expression on channels permitted detailed functional investigation of this channel. (rupress.org)
  • Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1. (bionity.com)
  • The CLC channel structure has not yet been resolved, however the structure of the CLC exchangers has been resolved by x-ray crystallography. (wikipedia.org)
  • Because the primary structure of the channels and exchangers are so similar, most assumptions about the structure of the channels are based on the structure established for the bacterial exchangers. (wikipedia.org)
  • In mammals, ClC-1, -2, -Ka/-Kb are plasma membrane Cl(-) channels, whereas ClC-3 through ClC-7 are 2Cl(-)/H+(-exchangers in endolysosomal membranes. (mdc-berlin.de)
  • Functionally, we demonstrate that the classic chloride channel inhibitor, 5-nitro-2-(3-phenylpropylamino)benzoic acid (NPPB), inhibited halide flux in cultured HASM cells. (sigmaaldrich.com)
  • However, for many years, muscle chloride channels resisted a detailed biophysical characterization. (rupress.org)
  • Publications] Uchida,S et al: 'Isolation and Characterization of kidney-specific CLC-K1 chloride channel gene promoter' Am.J.Physiol.274. (nii.ac.jp)
  • Isolation and characterization of kidney-specific CIC-K1 chloride channel gene promoter. (nii.ac.jp)
  • Each channel or exchanger is composed of two similar subunits-a dimer-each subunit containing one pore. (wikipedia.org)
  • Initially, cell excitability was mainly studied in neuronal axons, and in this particular cell compartment, electrical excitability is almost exclusively governed by cation channels. (rupress.org)
  • ClC channels conduct chloride (Cl - ) ions across cell membranes and thereby govern the electrical activity of muscle cells and certain neurons, the transport of fluid and electrolytes across epithelia, and the acidification of intracellular vesicles. (sciencemag.org)
  • This means that the chloride and the proton share a common pathway on the extracellular side, but diverge on the intracellular side. (wikipedia.org)
  • Crystal structures of wild-type and mutant Escherichia coli ClC channels bound to a monoclonal Fab fragment reveal three Cl - binding sites within the 15-angstrom neck of an hourglass-shaped pore. (sciencemag.org)
  • Researchers led by Professor Thomas Jentsch have now been able to show for the first time how the altered channels cause the disease. (innovations-report.com)
  • The team, led by Prof. Thomas Jentsch - a pioneer who discovered the first chloride channel family, including ClC-2, almost three decades ago, initially investigated all known aldosteronism-causing ClC-2 mutations in vitro. (innovations-report.com)
  • Recent findings of TMEM16B in photoreceptor terminals ( 10 ) and olfactory neuron cilia ( 11 ) suggest that calcium-activated chloride channels containing the TMEM16B subunit likely fulfill the negative and positive feedback regulation, respectively, in these sensory neurons. (pnas.org)
  • Publications] Matsumura.Y et al: 'Transcriptional regulation of aquaporin-2 water channel gene by CAUP' J.Am.Soc.Nephrol.8. (nii.ac.jp)
  • Transcriptional regulation of aquaporin-2 water channel gene by cAMP. (nii.ac.jp)
  • Some chloride channels are activated only by voltage (i.e., voltage-gated), while others are activated by Ca2+, other extracellular ligands, or pH. (wikipedia.org)
  • In 2018, Paris-based scientists around Prof. Maria-Christina Zennaro teamed up with colleagues from the Leibniz-Forschungsinstitut fuer Molekulare Pharmakologie (FMP) and the Max Delbrueck Center (MDC) as well as other scientists in Germany and the USA around Prof. Ute Scholl from the Berlin Institute of Health (BIH) and Charite, and found mutations in the ClC-2 chloride channel in patients affected by this syndrome. (innovations-report.com)
  • Bartter's syndrome, which is associated with renal salt wasting and hypokalemic alkalosis, is due to the defective transport of chloride ions and associated ions in the thick ascending loop of Henle. (bionity.com)