CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease
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Title
CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease
Authors
Keywords
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Journal
PHYSIOLOGICAL REVIEWS
Volume 98, Issue 3, Pages 1493-1590
Publisher
American Physiological Society
Online
2018-06-01
DOI
10.1152/physrev.00047.2017
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- Japanese Dent disease has a wider clinical spectrum than Dent disease in Europe/USA: genetic and clinical studies of 86 unrelated patients with low-molecular-weight proteinuria
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- Expanding the spectrum of megalencephalic leukoencephalopathy with subcortical cysts in two patients with GLIALCAM mutations
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- Novel brain expression of ClC-1 chloride channels and enrichment of CLCN1 variants in epilepsy
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- Clinical and molecular study of a new form of hereditary myotonia in Murrah water buffalo
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- Chloride channel 4 is required for nerve growth factor-induced TrkA signaling and neurite outgrowth in PC12 cells and cortical neurons
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- Acid-sensitive outwardly rectifying (ASOR) anion channels in human epithelial cells are highly sensitive to temperature and independent of ClC-3
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- CLCNKB mutations causing mild Bartter syndrome profoundly alter the pH and Ca2+ dependence of ClC-Kb channels
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- Regulation of ClC-2 gating by intracellular ATP
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- Myotonia Congenita Mutation Enhances the Degradation of Human CLC-1 Chloride Channels
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- Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction
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- Water access points and hydration pathways in CLC H+/Cl−transporters
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- Receptor-mediated endocytosis and endosomal acidification is impaired in proximal tubule epithelial cells of Dent disease patients
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- Molecular determinants of common gating of a ClC chloride channel
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- An optical assay of the transport activity of ClC-7
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- Manumycin A corrects aberrant splicing of Clcn1 in myotonic dystrophy type 1 (DM1) mice
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- Ultrasound-enhanced delivery of Morpholino with Bubble liposomes ameliorates the myotonia of myotonic dystrophy model mice
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- Lubiprostone Increases Small Intestinal Smooth Muscle Contractions Through a Prostaglandin E Receptor 1 (EP)-mediated Pathway
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- Differential regulation of a CLC anion channel by SPAK kinase ortholog-mediated multisite phosphorylation
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- ClC-3 is a candidate of the channel proteins mediating acid-activated chloride currents in nasopharyngeal carcinoma cells
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- Chloride in Vesicular Trafficking and Function
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- Lubiprostone activates CFTR, but not ClC-2, via the prostaglandin receptor (EP4)
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- Surprises from an Unusual CLC Homolog
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- On the Mechanism of Gating Charge Movement of ClC-5, a Human Cl−/H+ Antiporter
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- A Designed Inhibitor of a CLC Antiporter Blocks Function through a Unique Binding Mode
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- Lubiprostone Targets Prostanoid Signaling and Promotes Ion Transporter Trafficking, Mucus Exocytosis, and Contractility
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- May the Truth Be with You: Lubiprostone as EP Receptor Agonist/ClC-2 Internalizing “Inhibitor”
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- Functional Regulation of ClC-3 in the Migration of Vascular Smooth Muscle Cells
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- Intracellular β-Nicotinamide Adenine Dinucleotide Inhibits the Skeletal Muscle ClC-1 Chloride Channel
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- Dissecting a regulatory calcium-binding site of CLC-K kidney chloride channels
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- Adaptor Protein Complexes 1 and 3 Are Essential for Generation of Synaptic Vesicles from Activity-Dependent Bulk Endosomes
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- Accumulation of autofluorescent storage material in brain is accelerated by ischemia in chloride channel 3 gene-deficient mice
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- Relationship between membrane Cl−conductance and contractile endurance in isolated rat muscles
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- Sequential interaction of chloride and proton ions with the fast gate steer the voltage-dependent gating in ClC-2 chloride channels
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- Disease-causing mutations C277R and C277Y modify gating of human ClC-1 chloride channels in myotonia congenita
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- CLC-3 chloride channels moderate long-term potentiation at Schaffer collateral-CA1 synapses
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- Synergistic substrate binding determines the stoichiometry of transport of a prokaryotic H+/Cl− exchanger
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- A new explanation for recessive myotonia congenita: Exon deletions and duplications in CLCN1
- (2012) D. L. Raja Rayan et al. NEUROLOGY
- GlialCAM, a Protein Defective in a Leukodystrophy, Serves as a ClC-2 Cl− Channel Auxiliary Subunit
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- Molecular mechanism of proton transport in CLC Cl-/H+ exchange transporters
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- Mechanism of proton/substrate coupling in the heptahelical lysosomal transporter cystinosin
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- Fluoride resistance and transport by riboswitch-controlled CLC antiporters
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- Intracellular Proton Access in a Cl−/H+ Antiporter
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- Mutant GlialCAM Causes Megalencephalic Leukoencephalopathy with Subcortical Cysts, Benign Familial Macrocephaly, and Macrocephaly with Retardation and Autism
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- Pre-steady-state and reverse transport currents in the GABA transporter GAT1
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- Kinase activation of ClC-3 accelerates cytoplasmic condensation during mitotic cell rounding
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- Movement of hClC-1 C-termini during common gating and limits on their cytoplasmic location
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- The Coupled Proton Transport in the ClC-ec1 Cl−/H+ Antiporter
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- Anion- and Proton-Dependent Gating of ClC-4 Anion/Proton Transporter under Uncoupling Conditions
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- Activation of Volume Regulated Chloride Channels Protects Myocardium from Ischemia/reperfusion Damage in Second-window Ischemic Preconditioning
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- WNK3 is a Putative Chloride-sensing Kinase
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- Putting the pieces together
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- ClC-7 is a slowly voltage-gated 2Cl−/1H+-exchanger and requires Ostm1 for transport activity
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- OCRL controls trafficking through early endosomes via PtdIns4,5P2-dependent regulation of endosomal actin
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- Bartter syndrome in two sisters with a novel mutation of the CLCNKB gene, one with deafness
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- Severe Defects in Absorptive Ion Transport in Distal Colons of Mice That Lack ClC-2 Channels
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- Lack of association of variants of the renal salt reabsorption-related genes SLC12A3 and ClC-Kb and hypertension in Mongolian and Han populations in Inner Mongolia
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- Clcn4-2 genomic structure differs between the X locus in Mus spretus and the autosomal locus in Mus musculus: AT motif enrichment on the X
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- Chloride Channel (Clc)-5 Is Necessary for Exocytic Trafficking of Na+/H+Exchanger 3 (NHE3)
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- Extracellular Determinants of Anion Discrimination of the Cl−/H+Antiporter Protein CLC-5
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- Physiology and Pathophysiology of CLC-1: Mechanisms of a Chloride Channel Disease, Myotonia
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- Report of two Chinese patients suffering from CLCN7-related osteopetrosis and root dysplasia
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- Binding of ATP to the CBS domains in the C-terminal region of CLC-1
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- Relationships between resting conductances, excitability, and t-system ionic homeostasis in skeletal muscle
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- In-vivo administration of CLC-K kidney chloride channels inhibitors increases water diuresis in rats
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- ClC-2 Channels Regulate Neuronal Excitability, Not Intracellular Chloride Levels
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- Thyroid iodide efflux: a team effort?
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- Degradation of Alzheimer's amyloid fibrils by microglia requires delivery of ClC-7 to lysosomes
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- The murine goblet cell protein mCLCA3 is a zinc-dependent metalloprotease with autoproteolytic activity
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- Presynaptic CLC-3 determines quantal size of inhibitory transmission in the hippocampus
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- Screening for mutations in Spanish families with myotonia. Functional analysis of novel mutations in CLCN1 gene
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- A missense mutation in the skeletal muscle chloride channel 1 (CLCN1) as candidate causal mutation for congenital myotonia in a New Forest pony
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- A CLCN1 mutation in dominant myotonia congenita impairs the increment of chloride conductance during repetitive depolarization
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- Bartter- and Gitelman-like syndromes: salt-losing tubulopathies with loop or DCT defects
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- ClC-5 mutations associated with Dent’s disease: a major role of the dimer interface
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- Loss-of-function DNA sequence variant in the CLCNKA chloride channel implicates the cardio-renal axis in interindividual heart failure risk variation
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- ClC-2 regulates mucosal barrier function associated with structural changes to the villus and epithelial tight junction
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- A Critical Role for Chloride Channel-3 (CIC-3) in Smooth Muscle Cell Activation and Neointima Formation
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- Structure of a Slow CLC Cl−/H+Antiporter from a Cyanobacterium
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- CLC channels and transporters: Proteins with borderline personalities
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- Accessibility of the CLC-0 Pore to Charged Methanethiosulfonate Reagents
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- Lubiprostone ameliorates the cystic fibrosis mouse intestinal phenotype
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- Gene trapping identifies chloride channel 4 as a novel inducer of colon cancer cell migration, invasion and metastases
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- Identification of sites responsible for the potentiating effect of niflumic acid on ClC-Ka kidney chloride channels
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- Lubiprostone targets prostanoid EP4 receptors in ovine airways
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- ClC-3—A Granular Anion Transporter Involved in Insulin Secretion?
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- Functional Study of CLC-1 Mutants Expressed in Xenopus Oocytes Reveals that a C-terminal Region Thr891-Ser892-Thr893 is Responsible for the Effects of Protein Kinase C Activator
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- Unique gating properties ofC. elegansClC anion channel splice variants are determined by altered CBS domain conformation and the R-helix linker
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- Lubiprostone Activates Cl− Secretion via cAMP Signaling and Increases Membrane CFTR in the Human Colon Carcinoma Cell Line, T84
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- Voltage-dependent charge movement associated with activation of the CLC-5 2Cl−/1H+ exchanger
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- Analysis of CLCN2 as Candidate Gene for Megalencephalic Leukoencephalopathy with Subcortical Cysts
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- Inter-subunit communication and fast gate integrity are important for common gating in hClC-1
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- Gitelman syndrome due to p.A204T mutation in CLCNKB gene
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- ATP Induces Conformational Changes in the Carboxyl-terminal Region of ClC-5
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- Role of ClC-5 in Renal Endocytosis Is Unique among ClC Exchangers and Does Not Require PY-motif-dependent Ubiquitylation
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- Sorting Motifs of the Endosomal/Lysosomal CLC Chloride Transporters
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- The Late Endosomal ClC-6 Mediates Proton/Chloride Countertransport in Heterologous Plasma Membrane Expression
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- Molecular Interaction and Functional Regulation of ClC-3 by Ca2+/Calmodulin-dependent Protein Kinase II (CaMKII) in Human Malignant Glioma
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- Activation of Swelling-activated Chloride Current by Tumor Necrosis Factor-α Requires ClC-3-dependent Endosomal Reactive Oxygen Production
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- A cation counterflux supports lysosomal acidification
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- The yeast CLC protein counteracts vesicular acidification during iron starvation
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- Relaxing messages from the sarcolemma: Figure 1.
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- Chloride channels take center stage in a muscular drama
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- Chloride currents from the transverse tubular system in adult mammalian skeletal muscle fibers
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- Sarcolemmal-restricted localization of functional ClC-1 channels in mouse skeletal muscle
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- Proton block of the CLC-5 Cl−/H+exchanger
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- A regulatory calcium-binding site at the subunit interface of CLC-K kidney chloride channels
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- Clcn5 Knockout Mice Exhibit Novel Immunomodulatory Effects and Are More Susceptible to Dextran Sulfate Sodium-Induced Colitis
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- Functional Characterization of a ClC-2-Like Cl− Conductance in Surface Epithelial Cells of Rat Rectal Colon
- (2010) Akihiro Inagaki et al. JOURNAL OF MEMBRANE BIOLOGY
- Distinct Neuropathologic Phenotypes After Disrupting the Chloride Transport Proteins ClC-6 or ClC-7/Ostm1
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- ClC-2 Voltage-Gated Channels Constitute Part of the Background Conductance and Assist Chloride Extrusion
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- Permeant anions contribute to voltage dependence of ClC-2 chloride channel by interacting with the protopore gate
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- Lactateper seimproves the excitability of depolarized rat skeletal muscle by reducing the Cl−conductance
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- Direct endosomal acidification by the outwardly rectifying CLC-5 Cl−/H+exchanger
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- Barttin Activates ClC-K Channel Function by Modulating Gating
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- Osteogenic role of endosomal chloride channels in MC3T3-E1 cells
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- Design, function and structure of a monomeric ClC transporter
- (2010) Janice L. Robertson et al. NATURE
- Regulation of fast-spiking basket cell synapses by the chloride channel ClC-2
- (2010) Csaba Földy et al. NATURE NEUROSCIENCE
- Antibodies against ClC7 inhibit extracellular acidification-induced Cl− currents and bone resorption activity in mouse osteoclasts
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- Disruption of ClC-2 expression is associated with progressive neurodegeneration in aging mice
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- Dent's disease
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- CLC-b-Mediated NOFormula/H+ Exchange Across the Tonoplast of Arabidopsis Vacuoles
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- The proline 160 in the selectivity filter of the Arabidopsis NO3−/H+ exchanger AtCLCa is essential for nitrate accumulation in planta
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- The Arabidopsis vacuolar anion transporter, AtCLCc, is involved in the regulation of stomatal movements and contributes to salt tolerance
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- The G215R Mutation in the Cl−/H+-Antiporter ClC-7 Found in ADO II Osteopetrosis Does Not Abolish Function but Causes a Severe Trafficking Defect
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- Lysosomal Pathology and Osteopetrosis upon Loss of H+-Driven Lysosomal Cl- Accumulation
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- Structure of a Eukaryotic CLC Transporter Defines an Intermediate State in the Transport Cycle
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- Endosomal Chloride-Proton Exchange Rather Than Chloride Conductance Is Crucial for Renal Endocytosis
- (2010) G. Novarino et al. SCIENCE
- Role of NHERF and scaffolding proteins in proximal tubule transport
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- An Alternative Splicing Variant in Clcn7–/– Mice Prevents Osteopetrosis but Not Neural and Retinal Degeneration
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- Physiology and pathophysiology of ClC-K/barttin channels
- (2010) Christoph Fahlke Frontiers in Physiology
- Molecular Basis of DFNB73: Mutations of BSND Can Cause Nonsyndromic Deafness or Bartter Syndrome
- (2009) Saima Riazuddin et al. AMERICAN JOURNAL OF HUMAN GENETICS
- Chloride Channels: Often Enigmatic, Rarely Predictable
- (2009) Charity Duran et al. Annual Review of Physiology
- PIKfyve-dependent regulation of the Cl− channel ClC-2
- (2009) Fabian Klaus et al. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
- Proton Transport Pathway in the ClC Cl−/H+ Antiporter
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- Statins and fenofibrate affect skeletal muscle chloride conductance in rats by differently impairing ClC-1 channel regulation and expression
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- Suppression of Sulfonylurea- and Glucose-Induced Insulin Secretion In Vitro and In Vivo in Mice Lacking the Chloride Transport Protein ClC-3
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- The Granular Chloride Channel ClC-3 Is Permissive for Insulin Secretion
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- Identification and functional analysis of novel mutations of the CLCNKB gene in Chinese patients with classic Bartter syndrome
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- Substrate-driven conformational changes in ClC-ec1 observed by fluorine NMR
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- Conversion of the 2 Cl−/1 H+ antiporter ClC-5 in a NO3−/H+ antiporter by a single point mutation
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- Intracellular regulation of human ClC-5 by adenine nucleotides
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- The role of protons in fast and slow gating of the Torpedo chloride channel ClC-0
- (2009) Giovanni Zifarelli et al. EUROPEAN BIOPHYSICS JOURNAL WITH BIOPHYSICS LETTERS
- Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7
- (2009) Lena Wartosch et al. FASEB JOURNAL
- Cereblon is expressed in the retina and binds to voltage-gated chloride channels
- (2009) Bettina Hohberger et al. FEBS LETTERS
- CLC transport proteins in plants
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- Activation of Intestinal Cl− Secretion by Lubiprostone Requires the Cystic Fibrosis Transmembrane Conductance Regulator
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- Two novelCLCN2mutations accelerating chloride channel deactivation are associated with idiopathic generalized epilepsy
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- Isolation and Characterization of a High Affinity Peptide Inhibitor of ClC-2 Chloride Channels
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- The ClC-3 Cl−/H+Antiporter Becomes Uncoupled at Low Extracellular pH
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- Residues Important for Nitrate/Proton Coupling in Plant and Mammalian CLC Transporters
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- An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice
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- Comparison of regulated passive membrane conductance in action potential–firing fast- and slow-twitch muscle
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- Regulation of ClC-1 and KATPchannels in action potential–firing fast-twitch muscle fibers
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- Channel-like slippage modes in the human anion/proton exchanger ClC-4
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- Intracellular Proton-Transfer Mutants in a CLC Cl−/H+Exchanger
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- CLCNKB-T481S and essential hypertension in a Ghanaian population
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- Cardiac-specific, inducible ClC-3 gene deletion eliminates native volume-sensitive chloride channels and produces myocardial hypertrophy in adult mice
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- Voltage-dependent and -independent titration of specific residues accounts for complex gating of a ClC chloride channel by extracellular protons
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- Myotonia congenita in a large consanguineous Arab family: Insight into the clinical spectrum of carriers and double heterozygotes of a novel mutation in the chloride channelCLCN1gene
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- Retraction Note to: Retraction: Mutations in CLCN2 encoding a voltage-gated chloride channel are associated with idiopathic generalized epilepsies
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- No evidence for a role of CLCN2 variants in idiopathic generalized epilepsy
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