ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5. (Q37668096)

21 August 2017

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title

ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5. (English)

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21 August 2017

Leigh Wellhauser

1

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21 August 2017

Christina D'Antonio

series ordinal

2

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21 August 2017

Christine E Bear

series ordinal

3

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21 August 2017

publication date

5 January 2010

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21 August 2017

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21 August 2017

volume

460

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21 August 2017

issue

2

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21 August 2017

page(s)

543-557

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The ClC-5 chloride channel knock-out mouse - an animal model for Dent's disease.

21 August 2017

cites work

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https://api.crossref.org/works/10.1007%2FS00424-009-0769-5

Characterization of osteoclasts from patients harboring a G215R mutation in ClC-7 causing autosomal dominant osteopetrosis type II

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00424-009-0769-5

Molecular structure and physiological function of chloride channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00424-009-0769-5

CBS domains form energy-sensing modules whose binding of adenosine ligands is disrupted by disease mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00424-009-0769-5

A structural perspective on ClC channel and transporter function

21 January 2018

1 reference

12 December 2020

High citrate diet delays progression of renal insufficiency in the ClC-5 knockout mouse model of Dent's disease

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12 December 2020

Inactivation of muscle chloride channel by transposon insertion in myotonic mice

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12 December 2020

Myotonia caused by mutations in the muscle chloride channel gene CLCN1.

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12 December 2020

Development of electrical myotonia in the ADR mouse: role of chloride conductance in myotubes and neonatal animals.

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12 December 2020

Novel CLCN5 mutations in patients with Dent's disease result in altered ion currents or impaired exchanger processing

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12 December 2020

Dent's disease, a renal Fanconi syndrome with nephrocalcinosis and kidney stones, is associated with a microdeletion involving DXS255 and maps to Xp11.22.

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12 December 2020

cAMP-mediated inhibition of the epithelial brush border Na+/H+ exchanger, NHE3, requires an associated regulatory protein

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12 December 2020

ClC-5: a chloride channel with multiple roles in renal tubular albumin uptake.

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12 December 2020

An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice.

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12 December 2020

Nox2 and Rac1 regulate H2O2-dependent recruitment of TRAF6 to endosomal interleukin-1 receptor complexes

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12 December 2020

A common molecular basis for three inherited kidney stone diseases

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12 December 2020

Anion channels, including ClC-3, are required for normal neutrophil oxidative function, phagocytosis, and transendothelial migration

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12 December 2020

Characterization of Dent's disease mutations of CLC-5 reveals a correlation between functional and cell biological consequences and protein structure.

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12 December 2020

Mutations in the chloride channel gene, CLCNKB, cause Bartter's syndrome type III

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12 December 2020

Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man

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12 December 2020

A C-Terminal PDZ Motif in NHE3 Binds NHERF-1 and Enhances cAMP Inhibition of Sodium−Hydrogen Exchange

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12 December 2020

Barttin increases surface expression and changes current properties of ClC-K channels

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12 December 2020

Identification of a PY motif in the epithelial Na channel subunits as a target sequence for mutations causing channel activation found in Liddle syndrome

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12 December 2020

Functional and structural conservation of CBS domains from CLC chloride channels.

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12 December 2020

Secondary active transport mediated by a prokaryotic homologue of ClC Cl- channels

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12 December 2020

Two isoforms of a chloride channel predominantly expressed in thick ascending limb of Henle's loop and collecting ducts of rat kidney

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12 December 2020

Carboxy-terminal truncations modify the outer pore vestibule of muscle chloride channels

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12 December 2020

Linking receptor-mediated endocytosis and cell signaling: evidence for regulated intramembrane proteolysis of megalin in proximal tubule

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12 December 2020

Receptor-mediated endocytosis in renal proximal tubule

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12 December 2020

Characterization of renal chloride channel (CLCN5) mutations in Dent's disease

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12 December 2020

Albumin overload induces apoptosis in LLC-PK(1) cells

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12 December 2020

Identification of ClC-7 as a major pathway for Cl- movement in lysosomes

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12 December 2020

Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes

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12 December 2020

The beta2-adrenergic receptor interacts with the Na+/H+-exchanger regulatory factor to control Na+/H+ exchange

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12 December 2020

Proton gradient formation in early endosomes from proximal tubules

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12 December 2020

Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins

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12 December 2020

Mice lacking renal chloride channel, CLC-5, are a model for Dent's disease, a nephrolithiasis disorder associated with defective receptor-mediated endocytosis.

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12 December 2020

NHERF associations with sodium-hydrogen exchanger isoform 3 (NHE3) and ezrin are essential for cAMP-mediated phosphorylation and inhibition of NHE3

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12 December 2020

Nucleotide recognition by the cytoplasmic domain of the human chloride transporter ClC-5

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12 December 2020

Characterization of the bone phenotype in ClC-7-deficient mice

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12 December 2020

Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7.

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12 December 2020

Evidence for a prostaglandin-independent defect in chloride reabsorption in the loop of henle as a proximal cause of Bartter's syndrome

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12 December 2020

The vacuolar (H+)-ATPases--nature's most versatile proton pumps

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12 December 2020

Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules

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12 December 2020

Selective interaction of megalin with postsynaptic density-95 (PSD-95)-like membrane-associated guanylate kinase (MAGUK) proteins

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12 December 2020

N-terminal PDZ domain is required for NHERF dimerization

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12 December 2020

Megalin and cubilin: synergistic endocytic receptors in renal proximal tubule

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12 December 2020

Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption

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12 December 2020

Crystal structure of the cytoplasmic domain of the chloride channel ClC-0.

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12 December 2020

Cytoplasmic ATP inhibition of CLC-1 is enhanced by low pH

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12 December 2020

Gating the selectivity filter in ClC chloride channels

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12 December 2020

Intracellular regulation of human ClC-5 by adenine nucleotides

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12 December 2020

NHERF family and NHE3 regulation

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12 December 2020

Genome search for susceptibility loci of common idiopathic generalised epilepsies

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12 December 2020

CLCN2 variants in idiopathic generalized epilepsy

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12 December 2020

Involvement of helices at the dimer interface in ClC-1 common gating

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12 December 2020

Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen)

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12 December 2020

Functional evaluation of Dent's disease-causing mutations: implications for ClC-5 channel trafficking and internalization.

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12 December 2020

CLC-3 deficiency leads to phenotypes similar to human neuronal ceroid lipofuscinosis

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12 December 2020

Dent's disease; a familial proximal renal tubular syndrome with low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, metabolic bone disease, progressive renal failure and a marked male predominance.

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12 December 2020

Ion permeation through a Cl--selective channel designed from a CLC Cl-/H+ exchanger

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12 December 2020

Evidence for genetic heterogeneity in Dent's disease

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12 December 2020

Vitamin A responsive night blindness in Dent's disease

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12 December 2020

Molecular cloning of a chloride channel that is regulated by dehydration and expressed predominantly in kidney medulla

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12 December 2020

PKB and megalin determine the survival or death of renal proximal tubule cells

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12 December 2020

Endotoxin priming of neutrophils requires NADPH oxidase-generated oxidants and is regulated by the anion transporter ClC-3.

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12 December 2020

Intracellular Proton-Transfer Mutants in a CLC Cl − /H + Exchanger

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12 December 2020

Severely impaired urine-concentrating ability in mice lacking the CLC-K1 chloride channel.

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12 December 2020

The structure of the cytoplasmic domain of the chloride channel ClC-Ka reveals a conserved interaction interface

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12 December 2020

Chloride/proton antiporter activity of mammalian CLC proteins ClC-4 and ClC-5.

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12 December 2020

Cytoplasmic ATP-sensing domains regulate gating of skeletal muscle ClC-1 chloride channels

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12 December 2020

Altered Na+ channel activity and reduced Cl? conductance cause hyperexcitability in recessive generalized myotonia (becker)

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12 December 2020

Urine electrolyte, mineral, and protein excretion in NHERF-2 and NHERF-1 null mice

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12 December 2020

A chloride channel widely expressed in epithelial and non-epithelial cells

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12 December 2020

Coexpression of complementary fragments of ClC-5 and restoration of chloride channel function in a Dent's disease mutation

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12 December 2020

Regulated intramembrane proteolysis of megalin: linking urinary protein and gene regulation in proximal tubule?

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12 December 2020

Mutational analysis of CLC-5, cofilin and CLC-4 in patients with Dent's disease

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12 December 2020

Altered gating and regulation of a carboxy-terminal ClC channel mutant expressed in the Caenorhabditis elegans oocyte.

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12 December 2020

Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus

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12 December 2020

V-ATPase interacts with ARNO and Arf6 in early endosomes and regulates the protein degradative pathway

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12 December 2020

ClC-1 chloride channel mutations in myotonia congenita: variable penetrance of mutations shifting the voltage dependence

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12 December 2020

Chloride transport in the kidney: lessons from human disease and knockout mice.

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12 December 2020

Mutations in the chloride channel gene CLCNKB as a cause of classic Bartter syndrome

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12 December 2020

Chloride channel ClCN7 mutations are responsible for severe recessive, dominant, and intermediate osteopetrosis

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12 December 2020

Nonsense and missense mutations of the muscle chloride channel gene in patients with myotonia congenita

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12 December 2020

NHE3 inhibits PKA-dependent functional expression of CFTR by NHERF2 PDZ interactions.

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12 December 2020

Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders.

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12 December 2020

Altered GABAergic function accompanies hippocampal degeneration in mice lacking ClC-3 voltage-gated chloride channels

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12 December 2020

Identification of functionally important regions of the muscular chloride channel CIC-1 by analysis of recessive and dominant myotonic mutations

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12 December 2020

Pathogenesis and management of hypercalciuric nephrolithiasis

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12 December 2020

Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration

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12 December 2020

Functional evaluation of human ClC-2 chloride channel mutations associated with idiopathic generalized epilepsies

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12 December 2020

Cofilin interacts with ClC-5 and regulates albumin uptake in proximal tubule cell lines.

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12 December 2020

The COOH terminus of megalin regulates gene expression in opossum kidney proximal tubule cells

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12 December 2020

Altered polarity and expression of H+-ATPase without ultrastructural changes in kidneys of Dent's disease patients

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12 December 2020

Inhibition of skeletal muscle ClC-1 chloride channels by low intracellular pH and ATP.

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12 December 2020

Barttin is a Cl- channel beta-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion

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12 December 2020

Dent-2 disease: a mild variant of Lowe syndrome

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12 December 2020

Inhibition of Na+-H+ exchange impairs receptor-mediated albumin endocytosis in renal proximal tubule-derived epithelial cells from opossum

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12 December 2020

Physiological importance of endosomal acidification: potential role in proximal tubulopathies

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12 December 2020

Mutational analysis demonstrates that ClC-4 and ClC-5 directly mediate plasma membrane currents.

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12 December 2020

Novel OCRL1 mutations in patients with the phenotype of Dent disease.

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12 December 2020

Nedd4-2 functionally interacts with ClC-5: involvement in constitutive albumin endocytosis in proximal tubule cells

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12 December 2020

An internalization signal in ClC-5, an endosomal Cl-channel mutated in dent's disease.

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12 December 2020

Determinants of anion-proton coupling in mammalian endosomal CLC proteins

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12 December 2020

The muscle chloride channel ClC-1 is not directly regulated by intracellular ATP

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12 December 2020

Impaired acidification in early endosomes of ClC-5 deficient proximal tubule

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12 December 2020

Regulation of albumin endocytosis by PSD95/Dlg/ZO-1 (PDZ) scaffolds. Interaction of Na+-H+ exchange regulatory factor-2 with ClC-5.

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12 December 2020

ClC-3 chloride channels facilitate endosomal acidification and chloride accumulation

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12 December 2020

Internalization of proximal tubular type II Na-P(i) cotransporter by PTH: immunogold electron microscopy

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12 December 2020

Organelle acidification and disease

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12 December 2020

Specific association of megalin and the Na+/H+ exchanger isoform NHE3 in the proximal tubule

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12 December 2020

Human ClC-6 is a late endosomal glycoprotein that associates with detergent-resistant lipid domains

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12 December 2020

Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.

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12 December 2020

ATP inhibition of CLC-1 is controlled by oxidation and reduction

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12 December 2020

Electrophysiology of reactive oxygen production in signaling endosomes

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12 December 2020

ATP binding to the C terminus of the Arabidopsis thaliana nitrate/proton antiporter, AtCLCa, regulates nitrate transport into plant vacuoles

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12 December 2020

Leukoencephalopathy upon disruption of the chloride channel ClC-2.

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12 December 2020

The skeletal muscle chloride channel in dominant and recessive human myotonia

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12 December 2020

Nonsense and missense mutations in the muscular chloride channel gene Clc-1 of myotonic mice.

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12 December 2020

Proteinuria and growth factors in the development of tubulointerstitial injury and scarring in kidney disease

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12 December 2020

Cytokine activation of nuclear factor kappa B in vascular smooth muscle cells requires signaling endosomes containing Nox1 and ClC-3

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12 December 2020

Suppressed diluting segment reabsorption in Bartter's syndrome: studies in 1 patient and synthesis of literature data

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12 December 2020

ClC-5 Cl- -channel disruption impairs endocytosis in a mouse model for Dent's disease.

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12 December 2020

Mutation of BSND causes Bartter syndrome with sensorineural deafness and kidney failure

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12 December 2020

Genomic organization of the human muscle chloride channel CIC-1 and analysis of novel mutations leading to Becker-type myotonia.

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12 December 2020

The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes.

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12 December 2020

ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function

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12 December 2020

Functional characterization of recombinant human ClC-4 chloride channels in cultured mammalian cells

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12 December 2020

Separate ion pathways in a Cl-/H+ exchanger

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12 December 2020

Targeted inactivation of Npt2 in mice leads to severe renal phosphate wasting, hypercalciuria, and skeletal abnormalities

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12 December 2020

Active (9.6 s) and inactive (21 s) oligomers of NHE3 in microdomains of the renal brush border

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12 December 2020

Conversion of the 2 Cl(-)/1 H+ antiporter ClC-5 in a NO3(-)/H+ antiporter by a single point mutation

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12 December 2020

X-ray structure of a ClC chloride channel at 3.0 A reveals the molecular basis of anion selectivity

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12 December 2020

Functional characterization of renal chloride channel, CLCN5, mutations associated with Dent'sJapan disease.

1 reference

12 December 2020

10.1007/S00424-009-0769-5

Identifiers

DOI

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21 August 2017

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