Published in Cell Physiol Biochem on January 01, 2005
Missense mutations in a retinal pigment epithelium protein, bestrophin-1, cause retinitis pigmentosa. Am J Hum Genet (2009) 1.53
Localization studies of rare missense mutations in cystic fibrosis transmembrane conductance regulator (CFTR) facilitate interpretation of genotype-phenotype relationships. Hum Mutat (2008) 1.15
Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator. Br J Pharmacol (2008) 1.09
Therapy through chaperones: sense or antisense? Cystic fibrosis as a model disease. J Inherit Metab Dis (2006) 1.04
Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins. J Biol Chem (2010) 0.78
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations. N Engl J Med (2003) 4.43
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet (2013) 2.91
Transcription-dependent spatial arrangements of CFTR and adjacent genes in human cell nuclei. J Cell Biol (2004) 2.43
Somatic mosaicism for copy number variation in differentiated human tissues. Hum Mutat (2008) 2.30
Characterization of one- and two-photon excitation fluorescence resonance energy transfer microscopy. Methods (2003) 2.25
Podocin and MEC-2 bind cholesterol to regulate the activity of associated ion channels. Proc Natl Acad Sci U S A (2006) 2.16
Loss of TMEM16A causes a defect in epithelial Ca2+-dependent chloride transport. J Biol Chem (2009) 2.07
TMEM16A induces MAPK and contributes directly to tumorigenesis and cancer progression. Cancer Res (2012) 1.98
Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation. Chem Biol (2008) 1.97
Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers. J Physiol (2005) 1.97
Expression and function of epithelial anoctamins. J Biol Chem (2010) 1.95
TMEM16 proteins produce volume-regulated chloride currents that are reduced in mice lacking TMEM16A. J Biol Chem (2009) 1.89
Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression. Am J Physiol Cell Physiol (2006) 1.82
Calmodulin-dependent activation of the epithelial calcium-dependent chloride channel TMEM16A. FASEB J (2010) 1.78
A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein. J Biol Chem (2010) 1.75
Store-independent Orai1/3 channels activated by intracrine leukotriene C4: role in neointimal hyperplasia. Circ Res (2013) 1.68
Essential role for STIM1/Orai1-mediated calcium influx in PDGF-induced smooth muscle migration. Am J Physiol Cell Physiol (2010) 1.58
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Mol Cell Biol (2005) 1.58
Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms. Proc Natl Acad Sci U S A (2006) 1.56
Anoctamins are a family of Ca2+-activated Cl- channels. J Cell Sci (2012) 1.55
Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis. Trends Pharmacol Sci (2007) 1.55
Efficient intracellular processing of the endogenous cystic fibrosis transmembrane conductance regulator in epithelial cell lines. J Biol Chem (2004) 1.50
Orai1-mediated I (CRAC) is essential for neointima formation after vascular injury. Circ Res (2011) 1.50
Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones. Biochem J (2008) 1.49
Marginal costs of hospital-acquired conditions: information for priority-setting for patient safety programmes and research. J Health Serv Res Policy (2011) 1.48
Anoctamin 6 is an essential component of the outwardly rectifying chloride channel. Proc Natl Acad Sci U S A (2011) 1.46
Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells. Genes Dev (2007) 1.43
Activation of the unfolded protein response by deltaF508 CFTR. Am J Respir Cell Mol Biol (2008) 1.41
Bestrophin 1 and 2 are components of the Ca(2+) activated Cl(-) conductance in mouse airways. Biochim Biophys Acta (2008) 1.33
Chapter 22: Quantitation of protein-protein interactions: confocal FRET microscopy. Methods Cell Biol (2008) 1.32
The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70. Biochem J (2002) 1.31
Bestrophin-1 enables Ca2+-activated Cl- conductance in epithelia. J Biol Chem (2006) 1.31
Confocal FRET microscopy to measure clustering of ligand-receptor complexes in endocytic membranes. Biophys J (2003) 1.29
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis. Am J Respir Cell Mol Biol (2002) 1.26
Enhanced expression of ANO1 in head and neck squamous cell carcinoma causes cell migration and correlates with poor prognosis. PLoS One (2012) 1.26
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon. Gastroenterology (2004) 1.25
Mechanistic insight into control of CFTR by AMPK. J Biol Chem (2008) 1.21
Proteomic analysis of nasal cells from cystic fibrosis patients and non-cystic fibrosis control individuals: search for novel biomarkers of cystic fibrosis lung disease. Proteomics (2006) 1.21
A genetic screening strategy identifies novel regulators of the proteostasis network. PLoS Genet (2011) 1.19
Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR. Proc Natl Acad Sci U S A (2007) 1.17
Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport. J Biol Chem (2010) 1.15
Expression of voltage-gated potassium channels in human and mouse colonic carcinoma. Clin Cancer Res (2007) 1.15
Evaluation of the clinical significance of homB, a novel candidate marker of Helicobacter pylori strains associated with peptic ulcer disease. J Infect Dis (2008) 1.14
Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry. J Biol Chem (2003) 1.12
Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells. Am J Respir Cell Mol Biol (2009) 1.12
Restoration of W1282X CFTR activity by enhanced expression. Am J Respir Cell Mol Biol (2007) 1.10
Quantitative tomographic imaging of intermolecular FRET in small animals. Biomed Opt Express (2012) 1.10
Cl transport in complemented CF bronchial epithelial cells correlates with CFTR mRNA expression levels. Cell Physiol Biochem (2008) 1.10
Modulation of Ca2+-activated Cl- secretion by basolateral K+ channels in human normal and cystic fibrosis airway epithelia. Pediatr Res (2003) 1.09
No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. Am J Respir Cell Mol Biol (2007) 1.08
Antibodies for CFTR studies. J Cyst Fibros (2004) 1.06
The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response. J Biol Chem (2008) 1.06
The EF-hand Ca2+-binding protein p22 plays a role in microtubule and endoplasmic reticulum organization and dynamics with distinct Ca2+-binding requirements. Mol Biol Cell (2003) 1.05
Role of anoctamins in cancer and apoptosis. Philos Trans R Soc Lond B Biol Sci (2014) 1.04
Voltage-gated K+ channels support proliferation of colonic carcinoma cells. FASEB J (2006) 1.04
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology (2004) 1.04
Issues in confocal microscopy for quantitative FRET analysis. Microsc Res Tech (2006) 1.04
ER-localized bestrophin 1 activates Ca2+-dependent ion channels TMEM16A and SK4 possibly by acting as a counterion channel. Pflugers Arch (2009) 1.04
Excellent in vivo bystander activity of fludarabine phosphate against human glioma xenografts that express the escherichia coli purine nucleoside phosphorylase gene. Cancer Res (2004) 1.03
Purinergic P2Y6 receptors induce Ca2+ and CFTR dependent Cl- secretion in mouse trachea. Cell Physiol Biochem (2005) 1.03
CFTR and TMEM16A are separate but functionally related Cl- channels. Cell Physiol Biochem (2011) 1.02
Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis. J Biol Chem (2005) 1.01
Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54. J Mol Med (Berl) (2011) 1.00
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR. J Physiol (2014) 1.00
Rotavirus toxin NSP4 induces diarrhea by activation of TMEM16A and inhibition of Na+ absorption. Pflugers Arch (2011) 1.00
Biochemical methods to assess CFTR expression and membrane localization. J Cyst Fibros (2004) 1.00
Diagnostic and prognostic biomarker discovery strategies for autoimmune disorders. J Proteomics (2009) 0.99
Interactions among p22, glyceraldehyde-3-phosphate dehydrogenase and microtubules. Biochem J (2004) 0.99
Ablation of internalization signals in the carboxyl-terminal tail of the cystic fibrosis transmembrane conductance regulator enhances cell surface expression. J Biol Chem (2002) 0.99
The CFTR and ENaC debate: how important is ENaC in CF lung disease? Am J Physiol Lung Cell Mol Physiol (2012) 0.98
The induction of yes-associated protein expression after arterial injury is crucial for smooth muscle phenotypic modulation and neointima formation. Arterioscler Thromb Vasc Biol (2012) 0.97
AMPK controls epithelial Na(+) channels through Nedd4-2 and causes an epithelial phenotype when mutated. Pflugers Arch (2009) 0.97
Cl- interference with the epithelial Na+ channel ENaC. J Biol Chem (2005) 0.97
Cigarette smoke and CFTR: implications in the pathogenesis of COPD. Am J Physiol Lung Cell Mol Physiol (2013) 0.96
Eag1 and Bestrophin 1 are up-regulated in fast-growing colonic cancer cells. J Biol Chem (2008) 0.96
Non-invasive in vivo imaging of near infrared-labeled transferrin in breast cancer cells and tumors using fluorescence lifetime FRET. PLoS One (2013) 0.96
Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi. FEBS J (2013) 0.96
Activation of ion secretion via proteinase-activated receptor-2 in human colon. Am J Physiol Gastrointest Liver Physiol (2002) 0.96
NRF2 Oxidative Stress Induced by Heavy Metals is Cell Type Dependent. Curr Chem Genomics (2011) 0.96
Regulation of the epithelial Na+ channel by the protein kinase CK2. J Biol Chem (2008) 0.95
Role of KCNMA1 in breast cancer. PLoS One (2012) 0.94
Correction of the CF defect by curcumin: hypes and disappointments. Bioessays (2005) 0.94
Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein. Biochem Biophys Res Commun (2003) 0.94
Inhibition of protein kinase CK2 closes the CFTR Cl channel, but has no effect on the cystic fibrosis mutant deltaF508-CFTR. Cell Physiol Biochem (2009) 0.94
One- and two-photon fluorescence resonance energy transfer microscopy to establish a clustered distribution of receptor-ligand complexes in endocytic membranes. J Biomed Opt (2003) 0.94
Regulation of angiogenesis by hypoxia: the role of microRNA. Cell Mol Biol Lett (2012) 0.94
Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction. Chem Biol (2013) 0.93
Direct evidence that IFN-beta functions as a tumor-suppressor protein. J Interferon Cytokine Res (2002) 0.93