Family of chloride channels. Medical search. Frequent questions

The Calcium-Dependent Chloride Channel (Ca-ClC) proteins (or calcium-activated chloride channels (CaCCs), are heterogeneous groups of ligand-gated ion channels for chloride that have been identified in many epithelial and endothelial cell types as well as in smooth muscle cells. (wikipedia.org)
They include proteins from several structurally different families: chloride channel accessory (CLCA), bestrophin (BEST), and calcium-dependent chloride channel anoctamin (ANO or TMEM16) channels ANO1 is highly expressed in human gastrointestinal interstitial cells of Cajal, which are proteins which serve as intestinal pacemakers for peristalsis. (wikipedia.org)
In addition to their role as chloride channels some CLCA proteins function as adhesion molecules and may also have roles as tumour suppressors. (wikipedia.org)
We find that the electrophysiological and structural properties of these proteins indicate that they are new members of the ligand-gated ion channel superfamily. (nih.gov)
Jose joined the School of Biosciences in September 2016 as a Lecturer in Biological NMR and head of the NMR facility, where his research group specialises in the structural and functional studies of proteins able to insert in lipid membranes, forming pores and channels. (kent.ac.uk)
Our lab is interested in proteins with the ability to exists in a soluble form and in a membrane-bound form, where they usually form channels of pores. (kent.ac.uk)
CLCA proteins are thought to mediate calcium-activated chloride currents and are obviously involved in many other cellular functions, including mucus production,andhydration of mucus membranes of thenose, trachea and lungrespiratoryas well as theintestinal tractintestineandmodulation ofinnate immune responses. (fu-berlin.de)
Starting from native material or recombinant systems, we succeed with all types of membrane proteins: GPCRs, Ion Channels, Transporters, Receptors and Viral Proteins. (calixar.com)
also GABR3) is a 53 - 58 kDa member of the ligand-gated ion channel family of proteins. (rndsystems.com)
Chloride channels are various group of proteins that control fundamental cellular processes including stabilization of cell membrane potential, transepithelial transport, regulation of intracellular pH, and maintenance of cell volume. (prospecbio.com)
Ion channels are pore-forming proteins that allow the flow of ions across membranes, either plasma membranes, or the membranes of intracellular organelles [ 4 ]. (guidetoimmunopharmacology.org)
In addition to its functions as an electrolyte, chloride combines with hydrogen in the stomach to make hydrochloric acid, a powerful digestive enzyme that is responsible for the break down of proteins, absorption of other metallic minerals, and activation of intrinsic factor, which in turn absorbs vitamin B12. (t3db.ca)
Chloride-transporting proteins (CLC) play fundamental roles in many tissues in the plasma membrane as well as in intracellular membranes. (t3db.ca)
CLC proteins form a gene family that comprises nine members in mammals, at least four of which are involved in human genetic diseases. (t3db.ca)
This gene encodes a member of the tweety family of proteins. (modellab.cn)

Activation mechanism of the calcium-activated chloride channel TMEM16A revealed by cryo-EM" (PDF). (wikipedia.org)
mCLCA3 Does Not Contribute to Calcium-Activated Chloride Conductance in Murine Airways. (fu-berlin.de)
The Porcine Chloride Channel Calcium-Activated Family Member pCLCA4a Mirrors Lung Expression of the Human hCLCA4. (fu-berlin.de)
they function as bicarbonate permeable, calcium-activated chloride channels. (bvsalud.org)
Furthermore, in a study based on a molecular approach, Woodruff and colleagues discovered that IL-13 can stimulate the expression of chloride channel, calcium-activated, family member 1 (CLCA1), periostin, serine peptidase inhibitor, clade B (ovalbumin), and serpin family B member 2 (serpinB2), all of which are overexpressed in asthmatic individuals (8). (ees2010prague.org)

High potassium intake inhibited the basolateral 40 pS potassium channel (a Kir4.1/5.1 heterotetramer) in the distal convoluted tubule, decreased basolateral potassium conductance, and depolarized the distal convoluted tubule membrane in Kcnj10flox/flox mice, herein referred to as control mice. (elsevierpure.com)
Both glutathione S-transferase pi ( GSTpi ) (80%, 24/30 in tumor and 56.7%, 17/30 in the paired non-cancerous tissues) and cystic fibrosis transmembrane conductance regulator, ATP-binding cassette (sub-family C, member 7) ( CFTR ) (77%, 23/30 in tumor and 50%, 15/30 in the paired non-cancerous tissues) genes were prevalently hypermethylated in HCC as well as their neighboring non-cancerous tissues. (wjgnet.com)
Cystic fibrosis (CF) is caused by alterations in the CF transmembrane conductance regulator (CFTCR) gene that result in deranged sodium and chloride ion transport channels. (t3db.ca)
Probable large-conductance Ca(2+)-activated chloride channel. (cansar.ai)
The encoded protein functions as a calcium(2+)-activated large conductance chloride(-) channel, and may play a role in kidney tumorigenesis. (modellab.cn)

The channel transports negatively charged particles called chloride ions into and out of cells. (medlineplus.gov)
The transport of chloride ions helps control the movement of water in tissues, which is necessary for the production of thin, freely flowing mucus. (medlineplus.gov)
The CFTR protein also regulates the function of other channels, such as those that transport positively charged particles called sodium ions across cell membranes. (medlineplus.gov)
Mutations in the CFTR gene disrupt the function of the chloride channel, preventing the usual flow of chloride ions and water into and out of cells. (medlineplus.gov)
The resulting abnormal channel breaks down shortly after it is made, so it never reaches the cell membrane to transport chloride ions. (medlineplus.gov)
All of these changes prevent the channel from functioning properly, which impairs the transport of chloride ions and the movement of water into and out of cells. (medlineplus.gov)
Ryanodine is an ion channel, embedded in an internal membrane within the muscle cell, that surrounds a pocket of calcium ions. (the-scientist.com)
Chloride ions also have other important physiological roles. (t3db.ca)

Ca2+) (in) CaCCs that are known to occur in humans include: Accessories: CLCA1, CLCA2, CLCA3, and CLCA4 Anoctamins: ANO1 and ANO2 (potentially others) Bestrophins: BEST1, BEST2, BEST3, and BEST4 Voltage-gated ion channel Ion channel The anoctamins are only expressed in eukaryotes, with 10 members in vertebrates. (wikipedia.org)
Voltage-gated and ligand-gated are key ion channel genes that have been linked to distinct epilepsy phenotypes [ 9 ]. (hindawi.com)
Voltage-gated chloride channel. (calixar.com)
2011) The crystal structure of a voltage-gated sodium channel. (guidetoimmunopharmacology.org)
2005) Overview of molecular relationships in the voltage-gated ion channel superfamily. (guidetoimmunopharmacology.org)
Chloride Voltage-Gated Channel 5, CLCN5 or H(+)/Cl(-) exchange transporter 5 is encoded by the gene CLCN5 and is a member of the voltage-gated chloride channel (ClC) family. (antibodiesinc.com)
chloride voltage-gated channel 3 [Sour. (gsea-msigdb.org)
The role and the function of voltage-gated chloride channels of the CIC family and its defects leading to genetic diseases. (home.pl)

Avermectins interact with vertebrate and invertebrate GABA receptors and invertebrate glutamate-gated chloride channels. (nih.gov)
Among ligand-gated channel genes, the genes encoding gamma-aminobutyric acid (GABA) receptors are considered a hotspot for susceptibility of IGE because of the extensive distribution of GABA receptors in the central nervous system (CNS), their potential for postsynaptic inhibition, and regulation by therapeutically important antiepileptic drugs [ 10 ]. (hindawi.com)
Recently, multiple mutations in gamma 2 subunit of GABA receptors are discovered in two families which furnished the genetic evidence for possible role of GABA receptor system in epileptogenesis. (hindawi.com)
Gamma-aminobutyric acid (GABA) receptor is a ligand-gated chloride channel. (hindawi.com)
Malfunction of the gene encoding these subunits influences the expression, gating of the ion channels, and trafficking of GABA receptors to the cellular surface [ 13 ]. (hindawi.com)
During withdrawal from alcohol, the loss of GABA-A receptor stimulation causes a reduction in chloride flux and is associated with tremors, diaphoresis, tachycardia, anxiety, and seizures. (medscape.com)
GABA receptors are a family of chloride ion channels that mediate inhibitory neurotransmission. (medscape.com)
aminobutyric acid (GABA)-gated chloride channel in the insect central nervous system. (cdc.gov)
It is generally one of three beta-type subunits that contribute to a GABA-binding pentameric, postsynaptic chloride ion channel on neurons. (rndsystems.com)
Alternatively, it can apparently form a homopentameric channel that does not respond to GABA. (rndsystems.com)
GABA(A) receptors are pentameric complexes that function as ligand-gated chloride ion channels. (t3db.ca)
Considering that PTZ blocks the chloride channel coupled to the GABA A receptor complex, the present study aimed to analyze the possible modulation of GABAergic homeostasis within synaptic clefts in vitro . (hindawi.com)

CLIC1 inserts from the aqueous phase into phospholipid membranes, where it functions as an anion channel. (proteopedia.org)
The anion selectivity of PAC is determined by the positively charged lysine 319 residue on TM2, and replacing lysine 319 with a glutamate residue converts PAC to a cation-selective channel. (johnshopkins.edu)
3. Dutzler R, Campbell EB, Cadene M, Chait BT, MacKinnon R. (2002) X-ray structure of a ClC chloride channel at 3.0 A reveals the molecular basis of anion selectivity. (guidetoimmunopharmacology.org)
The chloride ion is an essential anion that the body needs for many critical functions. (t3db.ca)
Members of this family function as chloride anion channels. (modellab.cn)

Gao S, Xie L, Kawano T, Po MD, Guan S, Zhen M, Pirri JK, Alkema MJ (2015) The NCA sodium leak channel is required for persistent motor circuit activity that sustains locomotion. (yale.edu)
Kir4.1 in the distal convoluted tubule plays a key role in sensing plasma potassium and in modulating the thiazide-sensitive sodium-chloride cotransporter (NCC). (elsevierpure.com)
Tetrodotoxin exerts its effects by blocking voltage-activated sodium channels, terminating nerve conduction and muscle action potentials, leading to progressive paralysis and, in extreme cases, to death from respiratory failure. (cdc.gov)

Disease-causing mutations in the CFTR gene alter the production, structure, or stability of the chloride channel. (medlineplus.gov)
New research has greatly expanded knowledge of the specific mutations capable of causing cystic fibrosis (CF). Previous landmark research established that CF is caused by mutations in a gene, CFTR, encoding a critical channel protein that enables the movement of chloride in and out of cells. (nih.gov)
To better understand which CFTR mutations pose a risk of causing CF, researchers examined genetic records of almost 40,000 people diagnosed with CF-estimated to be more than half of the world population with the disease-for whom clinical measurements of CFTR chloride channel function had been recorded. (nih.gov)
Confining their analysis to 159 variants found in at least 0.01 percent (one in 10,000) of people with CF in the database they had assembled, the researchers tested how these CFTR mutations affect chloride transport when introduced into cells grown in the laboratory, and compared the results of those tests to clinical measurements of CF from their database. (nih.gov)
Moreover, the laboratory models of the different CFTR variants created through this research may promote better understanding of the physiology of the CFTR chloride channel, and may one day lead to improvements in CF patient care. (nih.gov)

Currently, ion channels (including ligand-gated ion channels) represent the second largest target for existing drugs after G protein-coupled receptors [ 5 ]. (guidetoimmunopharmacology.org)

Also, the chloride-bicarbonate exchanger biological transport protein relies on the chloride ion to increase the blood's capacity of carbon dioxide, in the form of the bicarbonate ion. (t3db.ca)
Description: The CLCN5 gene encodes the chloride channel Cl-/H+ exchanger ClC-5. (internationalshouldergroup.org)

CLIC1 is a member of the highly conserved class of chloride ion channels that exist in both soluble and integral membrane forms. (proteopedia.org)
At its binding site in the pore, chloride could interact with the ends of four helices that come from both sides of the membrane. (proteopedia.org)
This protein functions as a channel across the membrane of cells that produce mucus, sweat, saliva, tears, and digestive enzymes. (medlineplus.gov)
CLIC1 displays both nuclear and plasma membrane chloride ion channel activity. (prospecbio.com)

CLIC1 inserts into membranes and forms chloride ion channels. (prospecbio.com)
4. Hille B. (1986) Ionic channels: molecular pores of excitable membranes. (guidetoimmunopharmacology.org)

Chloride is a prominent negatively charged ion of the blood, where it represents 70% of the body's total negative ion content. (t3db.ca)

The whole-cell voltage clamp method ( 6 ) was chosen to measure swelling-induced chloride currents in isolated NIH 3T3 fibroblasts, colon carcinoma (CaCo) cells, and H9 cells, a human T cell lymphoma cell line (ATCC HTB 176). (biomedcentral.com)

charged amino acids on each end of the alpha helices could be part of the ion selectivity of this channel. (proteopedia.org)

CLCN5 is a proton-coupled chloride transporter. (antibodiesinc.com)

We investigated the swelling-induced chloride current in fibroblasts, which we demonstrated is closely related or identical to a cloned epithelial chloride channel, I Cln . (biomedcentral.com)

We show here a novel molecular mechanism by which antiviral drugs of the nucleoside analog family could lead to impairments of the kidney, bone marrow, gastrointestinal, and neuronal functions, and how these side effects could possibly be restricted by the presence of TDP or uridine. (biomedcentral.com)
1. Doyle DA, Morais Cabral J, Pfuetzner RA, Kuo A, Gulbis JM, Cohen SL, Chait BT, MacKinnon R. (1998) The structure of the potassium channel: molecular basis of K+ conduction and selectivity. (guidetoimmunopharmacology.org)

Three years after the initial discovery, they found that another protein structurally related to glutathione transferases, a chloride intracellular ion channel, CLIC-2, could also dampen the activity of the ryanodine receptor in the heart. (the-scientist.com)

It has a homodimeric structure with one pore per subunit, which creates an incredibly unique "double barreled" channel. (proteopedia.org)
cyclic nucleotide gated channel subunit. (gsea-msigdb.org)

A C. elegans messenger RNA expressed in Xenopus oocytes encodes an avermectin-sensitive glutamate-gated chloride channel. (nih.gov)
To elucidate the structure and properties of this channel, we used Xenopus oocytes for expression cloning of two functional complementary DNAs encoding an avermectin-sensitive glutamate-gated chloride channel. (nih.gov)

Many ion channels (such as most Na, K, Ca and some Cl channels) are gated by voltage but others (such as certain K and Cl channels, TRP channels, ryanodine receptors and IP 3 receptors) are relatively voltage-insensitive and are gated by second messengers and other intracellular and/or extracellular mediators. (guidetoimmunopharmacology.org)
This conference is focused on exploring the understudied druggable targets including ion channels, G protein coupled receptors, kinases, and network pharmacology. (druggablegenome.net)

chloride nucleotide-sensitive channel 1. (gsea-msigdb.org)

AZT and acyclovir block the swelling-dependent chloride current and the chloride flux in fibroblasts, and the regulatory volume decrease (RVD) and I Cl in H9 cells. (biomedcentral.com)
WNK kinases are a family of serine-threonine kinases that have been shown to play an essential role in the regulation of electrolyte homeostasis, and they are found in diverse epithelia throughout the body that are involved in chloride ion flux. (t3db.ca)

The CLIC family consists of seven members: CLIC1-5, p64, and parchorin. (proteopedia.org)
CLIC1 is the most commonly studied member of the CLIC family because it is expressed to some extent in most tissues and cell types that have been studied and is particularly highly expressed in muscle. (proteopedia.org)
This wide range of locations in the cell causes a plausible reason to assume that the CLIC chloride channel family participate in an equally wide variety of physiological processes. (proteopedia.org)
The CLIC family is defined by a COOH-terminal core segment of ~230 amino acids that are highly conserved among the family members. (proteopedia.org)
The Chloride Intracellular Channel (CLIC) has been linked with glioblastoma proliferative capacity and cardiac disease. (kent.ac.uk)

The ciliary epithelium, a bilayer of secretory neuroepithelial cells, is the site of synthesis of aqueous humor (inflow system), and it regulates its secretion by the coordinated action of a complex transport system that involves ion channels and active transporters. (yale.edu)
This gene encodes a member of the ClC family of chloride ion channels and ion transporters. (internationalshouldergroup.org)

During the past 17 years our research has focused on the CLCA gene family and its relevance in disease in humans and animals. (fu-berlin.de)
We hypothesize that members of the CLCA gene family play critical roles in these maladies and may be of interest for novel strategies for intervention and therapy. (fu-berlin.de)

Having pioneered the study of muscle physiology in mammals, she uncovered how ion channels enable muscle movement. (the-scientist.com)
2. Dunlop J, Bowlby M, Peri R, Vasilyev D, Arias R. (2008) High-throughput electrophysiology: an emerging paradigm for ion-channel screening and physiology. (guidetoimmunopharmacology.org)

Tmc1 and Tmc2 (TC#s 1.A.17.4.6 and 1.A.17.4.1, respectively) may play a role in hearing and are required for normal function of cochlear hair cells, possibly as Ca2+ channels or Ca2+ channel subunits (see also family TC# 1.A.82). (wikipedia.org)

CLC chloride channels: correlating structure with function. (proteopedia.org)
These channels are necessary for the normal function of organs such as the lungs and pancreas. (medlineplus.gov)
Chloride ion channels control several cellular processes required for the normal function of the cell, and growing evidence supports the role of this channels in the development of different types of cancer. (kent.ac.uk)

Electrophysiological, fluorescence optical, and volume measurements were made to determine the effect of nucleoside analogs on the swelling-dependent chloride current (I Cl ) in NIH 3T3 fibroblasts and in human T cell lymphoma (H9) cells and the cAMP-dependent chloride current in CaCo cells. (biomedcentral.com)

The CIC channels are present in different tissues and play a role in transmembrane potential stabilization, transepithelial transport, cell volume regulation, acidification of intracellular organelles. (home.pl)

Tetrodotoxin is a heat-stable and acid-stable, nonprotein, alkaloid toxin found in many species of the fish family Tetraodontidae (puffer fish) as well as in certain gobies, amphibians, invertebrates, and the blue-ringed octopus ( 2 ). (cdc.gov)

For electrical measurements in H9 cells, glass cover slips were coated with a 0.25% collagen solution (type I). All experiments were carried out at room temperature (20-22°C). Bath and pipette solutions were chosen to enable chloride current measurements. (biomedcentral.com)
The ciliary epithelium, a bilayer of secretory neuroepithelial cells, is the site of synthesis of aqueous humor (inflow system), and regulates its secretion by the coordinated action of a complex transport system involving ion channels and active ion secretion, from the stromal to the aqueous-humor surface, followed by osmotic water movement. (yale.edu)
The proton-activated chloride channel (PAC) is active across a wide range of mammalian cells and is involved in acid-induced cell death and tissue injury 1-3 . (johnshopkins.edu)

CLIC1 channel activity depends on the pH. (prospecbio.com)

On average, an adult human body contains approximately 115 grams of chloride, making up about 0.15% of total body weight. (t3db.ca)

Mutations in ion channel genes have been identified as a common cause of IGE. (hindawi.com)

aldo-keto reductase family 1 member A1. (gsea-msigdb.org)
NCDs have far reaching negative externalities not only through health of the affected individual, but losses incurred to family members, society and country due to productivity loss and prolonged care and treatment needed for a member with NCDs. (who.int)

Therefore, when considering having children, couples with CF in one of their families will often pursue genetic testing and counseling to determine the likelihood that one of their children will have the disease. (nih.gov)
The genetic defects of CIC-1 chloride channel lead to myotonias, the defect in CIC-5 channel to the formation of stones in kidney, while the defect in CIC-Kb channel leads to the Bartter's syndrome. (home.pl)
Recognition of a single-gene disorder as causal for a patient's 'multiple sclerosis-like' phenotype is critically important for accurate direction of patient management, and evokes broader genetic counselling implications for affected families. (medscape.com)

6][11] It belongs to the avermectin family of medications. (abovetopsecret.com)

Our group investigates different CLCA family members in humans, mice, horses and pigs to gain a better understanding of their roles in normal and diseased tissues. (fu-berlin.de)

family with sequence similarity 133 mem. (gsea-msigdb.org)
family with sequence similarity 210 me. (gsea-msigdb.org)

Anatomy25

Organisms15

Diseases7

Chemicals and Drugs153

Analytical, Diagnostic and Therapeutic Techniques and Equipment13

Phenomena and Processes38

Disciplines and Occupations3

Technology, Industry, Agriculture2

Information Science3

Proteins15

Calcium-activated5

Conductance5

Ions8

Voltage gated8

GABA12

Anion5

Sodium3

CFTR5

Ligand-gated io1

Exchanger2

Membrane4

Membranes2

Negatively charged1

Currents1

Helices1

CLCN51

Epithelial1

Molecular2

Structurally1

Subunit2

Xenopus2

Receptors2

Nucleotide1

Flux2

CLIC5

Transporters2

Gene Family2

Physiology2

Subunits1

Function3

Analogs1

Intracellular organelles1

Toxin1

Cells3

Activity1

Human1

Genes1

Member2

Genetic3

Avermectin1

Tissues1

Sequence similarity2