Published in Proc Natl Acad Sci U S A on July 08, 1997
High-level expression, functional reconstitution, and quaternary structure of a prokaryotic ClC-type chloride channel. J Gen Physiol (1999) 2.12
Elimination of the slow gating of ClC-0 chloride channel by a point mutation. J Gen Physiol (1999) 1.89
A serine residue in ClC-3 links phosphorylation-dephosphorylation to chloride channel regulation by cell volume. J Gen Physiol (1999) 1.61
Reorientation of aquaporin-1 topology during maturation in the endoplasmic reticulum. Mol Biol Cell (2000) 1.42
Gating competence of constitutively open CLC-0 mutants revealed by the interaction with a small organic Inhibitor. J Gen Physiol (2003) 1.41
Cysteine modification of a putative pore residue in ClC-0: implication for the pore stoichiometry of ClC chloride channels. J Gen Physiol (2000) 1.18
Integration of Shaker-type K+ channel, KAT1, into the endoplasmic reticulum membrane: synergistic insertion of voltage-sensing segments, S3-S4, and independent insertion of pore-forming segments, S5-P-S6. Proc Natl Acad Sci U S A (2001) 1.06
The ClC-3 Cl-/H+ antiporter becomes uncoupled at low extracellular pH. J Biol Chem (2009) 1.01
Evidence for the intracellular location of chloride channel (ClC)-type proteins: co-localization of ClC-6a and ClC-6c with the sarco/endoplasmic-reticulum Ca2+ pump SERCA2b. Biochem J (1998) 0.97
ClC chloride channels. Genome Biol (2001) 0.95
Modification of N-glycosylation sites allows secretion of bacterial chondroitinase ABC from mammalian cells. J Biotechnol (2009) 0.90
Novel mutations of CLCN7 cause autosomal dominant osteopetrosis type II (ADO-II) and intermediate autosomal recessive osteopetrosis (IARO) in Chinese patients. Osteoporos Int (2015) 0.85
Human ClC-6 is a late endosomal glycoprotein that associates with detergent-resistant lipid domains. PLoS One (2007) 0.83
Characteristics of ClC7 Cl- channels and their inhibition in mutant (G215R) associated with autosomal dominant osteopetrosis type II in native osteoclasts and hClcn7 gene-expressing cells. Pflugers Arch (2009) 0.78
The ClC-3 chloride channel and osmoregulation in the European sea bass, Dicentrarchus labrax. J Comp Physiol B (2013) 0.77
Expression and targeting to the plasma membrane of xClC-K, a chloride channel specifically expressed in distinct tubule segments of Xenopus laevis kidney. Biochem J (1999) 0.76
Unidirectionally thoracoscopic resection of lingual segment of the left upper pulmonary lobe. J Thorac Dis (2014) 0.75
Antibodies against ClC7 inhibit extracellular acidification-induced Cl⁻ currents and bone resorption activity in mouse osteoclasts. Naunyn Schmiedebergs Arch Pharmacol (2011) 0.75
Involvement of the L-Type Amino Acid Transporter Lat2 in the Transport of 3,3'-Diiodothyronine across the Plasma Membrane. Eur Thyroid J (2015) 0.75
A simple method for displaying the hydropathic character of a protein. J Mol Biol (1982) 150.65
PHD: predicting one-dimensional protein structure by profile-based neural networks. Methods Enzymol (1996) 9.10
Acetylcholine receptor channel structure probed in cysteine-substitution mutants. Science (1992) 5.61
Topogenic signals in integral membrane proteins. Eur J Biochem (1988) 5.16
Transmembrane helices predicted at 95% accuracy. Protein Sci (1995) 5.05
Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes. Nature (1990) 4.55
Topology prediction for helical transmembrane proteins at 86% accuracy. Protein Sci (1996) 4.50
The skeletal muscle chloride channel in dominant and recessive human myotonia. Science (1992) 4.28
Gating of the voltage-dependent chloride channel CIC-0 by the permeant anion. Nature (1995) 4.27
A common molecular basis for three inherited kidney stone diseases. Nature (1996) 4.26
Prediction of transmembrane segments in proteins utilising multiple sequence alignments. J Mol Biol (1994) 4.07
Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel. Nature (1991) 3.62
Two physically distinct pores in the dimeric ClC-0 chloride channel. Nature (1996) 3.58
Homodimeric architecture of a ClC-type chloride ion channel. Nature (1996) 3.47
Regions involved in the opening of CIC-2 chloride channel by voltage and cell volume. Nature (1993) 3.27
Determination of the distance between the oligosaccharyltransferase active site and the endoplasmic reticulum membrane. J Biol Chem (1993) 3.02
Inactivation of muscle chloride channel by transposon insertion in myotonic mice. Nature (1991) 2.74
Predicting the topology of eukaryotic membrane proteins. Eur J Biochem (1993) 2.73
Molecular basis of Thomsen's disease (autosomal dominant myotonia congenita). Nat Genet (1993) 2.56
Heteromultimeric CLC chloride channels with novel properties. Proc Natl Acad Sci U S A (1996) 2.28
Purification, reconstitution, and subunit composition of a voltage-gated chloride channel from Torpedo electroplax. Biochemistry (1994) 2.21
Two highly homologous members of the ClC chloride channel family in both rat and human kidney. Proc Natl Acad Sci U S A (1994) 2.17
Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen). EMBO J (1994) 2.08
Side-chain accessibilities in the pore of a K+ channel probed by sulfhydryl-specific reagents after cysteine-scanning mutagenesis. Biophys J (1995) 2.06
Structure of the NMDA receptor channel M2 segment inferred from the accessibility of substituted cysteines. Neuron (1996) 2.05
Molecular dissection of gating in the ClC-2 chloride channel. EMBO J (1997) 1.94
K+ pore structure revealed by reporter cysteines at inner and outer surfaces. Neuron (1995) 1.91
Properties of voltage-gated chloride channels of the ClC gene family. J Physiol (1995) 1.82
Membrane topology of a cysteine-less mutant of human P-glycoprotein. J Biol Chem (1995) 1.75
Structure of the sodium channel pore revealed by serial cysteine mutagenesis. Proc Natl Acad Sci U S A (1996) 1.67
Identification of acetylcholine receptor channel-lining residues in the M1 segment of the alpha-subunit. Biochemistry (1995) 1.64
The topological analysis of integral cytoplasmic membrane proteins. J Membr Biol (1993) 1.63
Alteration of GABAA receptor function following gene transfer of the CLC-2 chloride channel. Neuron (1996) 1.56
Membrane topology of the human Na+/glucose cotransporter SGLT1. J Biol Chem (1996) 1.54
A former amino terminal signal sequence engineered to an internal location directs translocation of both flanking protein domains. J Cell Biol (1985) 1.54
Identification of cystic fibrosis transmembrane conductance regulator channel-lining residues in and flanking the M6 membrane-spanning segment. Biophys J (1996) 1.54
Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (1994) 1.51
Subunit stoichiometry of human muscle chloride channels. J Gen Physiol (1997) 1.47
Topology of the Glut 1 glucose transporter deduced from glycosylation scanning mutagenesis. J Biol Chem (1994) 1.45
Two isoforms of a chloride channel predominantly expressed in thick ascending limb of Henle's loop and collecting ducts of rat kidney. J Biol Chem (1994) 1.42
Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion. J Biol Chem (1994) 1.42
Asparagine-linked oligosaccharides are localized to single extracytosolic segments in multi-span membrane glycoproteins. Biochem J (1994) 1.39
A family of putative chloride channels from Arabidopsis and functional complementation of a yeast strain with a CLC gene disruption. J Biol Chem (1996) 1.38
Systematic sequencing of the Escherichia coli genome: analysis of the 2.4-4.1 min (110,917-193,643 bp) region. Nucleic Acids Res (1994) 1.36
The GEF1 gene of Saccharomyces cerevisiae encodes an integral membrane protein; mutations in which have effects on respiration and iron-limited growth. Mol Gen Genet (1993) 1.24
Probing the transmembrane topology of cyclic nucleotide-gated ion channels with a gene fusion approach. Proc Natl Acad Sci U S A (1995) 1.14
Amino-terminal assembly of human P-glycoprotein at the endoplasmic reticulum is directed by cooperative actions of two internal sequences. J Biol Chem (1993) 1.12
Construction of defined polytopic integral transmembrane proteins. The role of signal and stop transfer sequence permutations. J Biol Chem (1988) 1.07
The transmembrane topology of the amino terminus of the alpha subunit of the nicotinic acetylcholine receptor. J Biol Chem (1991) 1.06
Biogenesis and transmembrane topology of the CHIP28 water channel at the endoplasmic reticulum. J Cell Biol (1994) 1.02
A novel integration signal that is composed of two transmembrane segments is required to integrate the Neurospora plasma membrane H(+)-ATPase into microsomes. J Biol Chem (1995) 1.01
Transmembrane orientation and topogenesis of the third and fourth membrane-spanning regions of human P-glycoprotein (MDR1). Cancer Res (1994) 0.96
Expression of fusion proteins of the nicotinic acetylcholine receptor from mammalian muscle identifies the membrane-spanning regions in the alpha and delta subunits. J Cell Biol (1992) 0.87
Cl- channels in basolateral renal medullary vesicles XI. rbClC-Ka cDNA encodes basolateral MTAL Cl- channels. Am J Physiol (1996) 0.83
KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness. Cell (1999) 4.52
Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man. Cell (2001) 4.36
The skeletal muscle chloride channel in dominant and recessive human myotonia. Science (1992) 4.28
Gating of the voltage-dependent chloride channel CIC-0 by the permeant anion. Nature (1995) 4.27
A common molecular basis for three inherited kidney stone diseases. Nature (1996) 4.26
A potassium channel mutation in neonatal human epilepsy. Science (1998) 4.17
A constitutively open potassium channel formed by KCNQ1 and KCNE3. Nature (2000) 3.75
Barttin is a Cl- channel beta-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion. Nature (2001) 3.69
Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel. Nature (1991) 3.62
A chloride channel widely expressed in epithelial and non-epithelial cells. Nature (1992) 3.60
Two physically distinct pores in the dimeric ClC-0 chloride channel. Nature (1996) 3.58
Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus. Neuron (2001) 3.51
Regions involved in the opening of CIC-2 chloride channel by voltage and cell volume. Nature (1993) 3.27
Moderate loss of function of cyclic-AMP-modulated KCNQ2/KCNQ3 K+ channels causes epilepsy. Nature (1998) 2.96
ClC-5 Cl- -channel disruption impairs endocytosis in a mouse model for Dent's disease. Nature (2000) 2.79
KCNQ5, a novel potassium channel broadly expressed in brain, mediates M-type currents. J Biol Chem (2000) 2.75
Inactivation of muscle chloride channel by transposon insertion in myotonic mice. Nature (1991) 2.74
Disruption of KCC2 reveals an essential role of K-Cl cotransport already in early synaptic inhibition. Neuron (2001) 2.71
KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway. Proc Natl Acad Sci U S A (2000) 2.45
Concentration and pH dependence of skeletal muscle chloride channel ClC-1. J Physiol (1996) 2.37
ClC-5, the chloride channel mutated in Dent's disease, colocalizes with the proton pump in endocytotically active kidney cells. Proc Natl Acad Sci U S A (1998) 2.34
Low single channel conductance of the major skeletal muscle chloride channel, ClC-1. Biophys J (1994) 2.28
Heteromultimeric CLC chloride channels with novel properties. Proc Natl Acad Sci U S A (1996) 2.28
Two highly homologous members of the ClC chloride channel family in both rat and human kidney. Proc Natl Acad Sci U S A (1994) 2.17
Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen). EMBO J (1994) 2.08
Mutations in the a3 subunit of the vacuolar H(+)-ATPase cause infantile malignant osteopetrosis. Hum Mol Genet (2000) 2.04
Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions. J Gen Physiol (1998) 2.02
Cloning and functional expression of rat CLC-5, a chloride channel related to kidney disease. J Biol Chem (1995) 2.00
Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the CIC-1 chloride channel. Neuron (1995) 1.94
Molecular dissection of gating in the ClC-2 chloride channel. EMBO J (1997) 1.94
Temperature dependence of fast and slow gating relaxations of ClC-0 chloride channels. J Gen Physiol (1997) 1.92
Completely functional double-barreled chloride channel expressed from a single Torpedo cDNA. Proc Natl Acad Sci U S A (1991) 1.91
Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption. EMBO J (2001) 1.86
Inhibition of KCNQ1-4 potassium channels expressed in mammalian cells via M1 muscarinic acetylcholine receptors. J Physiol (2000) 1.79
Mutational analysis demonstrates that ClC-4 and ClC-5 directly mediate plasma membrane currents. J Biol Chem (1999) 1.78
Molecular physiology of voltage-gated chloride channels. Physiol Rev (1994) 1.63
Pores formed by single subunits in mixed dimers of different CLC chloride channels. J Biol Chem (2000) 1.60
Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel. Proc Natl Acad Sci U S A (2001) 1.57
Alteration of GABAA receptor function following gene transfer of the CLC-2 chloride channel. Neuron (1996) 1.56
Surface expression and single channel properties of KCNQ2/KCNQ3, M-type K+ channels involved in epilepsy. J Biol Chem (2000) 1.53
Analysis of a protein region involved in permeation and gating of the voltage-gated Torpedo chloride channel ClC-0. J Physiol (1997) 1.50
Pathophysiological mechanisms of dominant and recessive KVLQT1 K+ channel mutations found in inherited cardiac arrhythmias. Hum Mol Genet (1997) 1.48
ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family. FEBS Lett (1995) 1.47
Evidence for coupled transport of bicarbonate and sodium in cultured bovine corneal endothelial cells. J Membr Biol (1984) 1.42
Characterization of the hyperpolarization-activated chloride current in dissociated rat sympathetic neurons. J Physiol (1998) 1.39
A family of putative chloride channels from Arabidopsis and functional complementation of a yeast strain with a CLC gene disruption. J Biol Chem (1996) 1.38
Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCN1) leading to myotonia. Am J Hum Genet (1995) 1.34
Golgi localization and functionally important domains in the NH2 and COOH terminus of the yeast CLC putative chloride channel Gef1p. J Biol Chem (1998) 1.33
Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders. Hum Mol Genet (1997) 1.27
Chloride dependence of hyperpolarization-activated chloride channel gates. J Physiol (1999) 1.26
Kidney epithelial cells of monkey origin (BSC-1) express a sodium bicarbonate cotransport. Characterization by 22Na+ flux measurements. J Biol Chem (1985) 1.24
Inward rectification in ClC-0 chloride channels caused by mutations in several protein regions. J Gen Physiol (1997) 1.24
ClC-1 chloride channel mutations in myotonia congenita: variable penetrance of mutations shifting the voltage dependence. Hum Mol Genet (1998) 1.20
Mutations in CAV3 cause mechanical hyperirritability of skeletal muscle in rippling muscle disease. Nat Genet (2001) 1.20
Independent gating of single pores in CLC-0 chloride channels. Biophys J (1997) 1.20
Genomic organization of the human muscle chloride channel CIC-1 and analysis of novel mutations leading to Becker-type myotonia. Hum Mol Genet (1994) 1.18
An internalization signal in ClC-5, an endosomal Cl-channel mutated in dent's disease. J Biol Chem (2000) 1.16
A bicarbonate-dependent process inhibitable by disulfonic stilbenes and a Na+/H+ exchange mediate 22Na+ uptake into cultured bovine corneal endothelium. J Biol Chem (1985) 1.16
Determinants of slow gating in ClC-0, the voltage-gated chloride channel of Torpedo marmorata. Am J Physiol (1998) 1.13
Characterization of renal chloride channel (CLCN5) mutations in Dent's disease. J Am Soc Nephrol (2000) 1.11
Kinetic properties of the sodium bicarbonate (carbonate) symport in monkey kidney epithelial cells (BSC-1). Interactions between Na+, HCO-3, and pH. J Biol Chem (1986) 1.10
Nonsense and missense mutations in the muscular chloride channel gene Clc-1 of myotonic mice. J Biol Chem (1994) 1.09
Idiopathic low molecular weight proteinuria associated with hypercalciuric nephrocalcinosis in Japanese children is due to mutations of the renal chloride channel (CLCN5). J Clin Invest (1997) 1.08
Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle. J Biol Chem (1994) 1.08
Localization and induction by dehydration of ClC-K chloride channels in the rat kidney. Am J Physiol (1997) 1.05
Functional and structural analysis of ClC-K chloride channels involved in renal disease. J Biol Chem (2000) 1.04
Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1. J Biol Chem (1997) 1.02
Novel muscle chloride channel (CLCN1) mutations in myotonia congenita with various modes of inheritance including incomplete dominance and penetrance. Neurology (1998) 0.97
The regulation of intracellular pH in monkey kidney epithelial cells (BSC-1). Roles of Na+/H+ antiport, Na+-HCO3(-)-(NaCO3-) symport, and Cl-/HCO3- exchange. J Biol Chem (1986) 0.93
Electrical properties of sodium bicarbonate symport in kidney epithelial cells (BSC-1). Am J Physiol (1986) 0.92
From tonus to tonicity: physiology of CLC chloride channels. J Am Soc Nephrol (2000) 0.92
CLC chloride channels in Caenorhabditis elegans. J Biol Chem (1999) 0.92
Kidney-specific upregulation of vitamin D3 target genes in ClC-5 KO mice. Kidney Int (2006) 0.91
Detection and differentiation of sensorineural hearing loss in mice using auditory steady-state responses and transient auditory brainstem responses. Neuroscience (2007) 0.89
Evidence for genetic homogeneity in autosomal recessive generalised myotonia (Becker). J Med Genet (1993) 0.88
Molecular basis of epithelial Cl channels. J Membr Biol (1995) 0.87
Tissue distribution and subcellular localization of the ClC-5 chloride channel in rat intestinal cells. Am J Physiol Cell Physiol (2001) 0.87
Trinity of cation channels. Nature (1994) 0.86
Effect of bicarbonate, pH, methazolamide and stilbenes on the intracellular potentials of cultured bovine corneal endothelial cells. J Membr Biol (1984) 0.84
Electrogenic sodium-bicarbonate symport in cultured corneal endothelial cells. Pflugers Arch (1985) 0.83
Interactions of pH and K+ conductance in cultured bovine retinal pigment epithelial cells. Am J Physiol (1986) 0.83
Molecular diagnosis of McArdle disease: revised genomic structure of the myophosphorylase gene and identification of a novel mutation. Hum Mutat (1998) 0.83
Regulation of intracellular pH in cultured bovine retinal pigment epithelial cells. Pflugers Arch (1988) 0.81
Anion dependence of electrical effects of bicarbonate and sodium on cultured bovine corneal endothelial cells. Pflugers Arch (1985) 0.81
ClC-7 expression levels critically regulate bone turnover, but not gastric acid secretion. Bone (2013) 0.80
Functional characterization of renal chloride channel, CLCN5, mutations associated with Dent'sJapan disease. Kidney Int (1998) 0.79
Neurological diseases caused by ion-channel mutations. Curr Opin Neurobiol (2000) 0.78
Ion transport mechanisms in cultured bovine corneal endothelial cells. Curr Eye Res (1985) 0.76
Response of the intracellular potentials of cultured bovine lens cells to ions and inhibitors. Exp Eye Res (1985) 0.76
Molecular physiology of renal chloride channels. Curr Opin Nephrol Hypertens (1998) 0.75
The calcium antagonist nisoldipine stimulates the electrolyte transport of the isolated frog skin. Biochem Pharmacol (1984) 0.75
Cell culture of bovine corneal endothelial cells and its application to transport studies. Methods Enzymol (1990) 0.75
Chloride channel 2 gene (Clc2) maps to chromosome 16 of the mouse, extending a region of conserved synteny with human chromosome 3q. Genet Res (1995) 0.75
KCNQ2, the first gene found to be mutated in human generalized idiopathic epilepsy. Pathol Biol (Paris) (1998) 0.75