Published in J Biol Chem on December 18, 2006
The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology. Physiol Rev (2012) 1.95
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner. J Biol Chem (2012) 1.80
G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects. J Gen Physiol (2007) 1.64
The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis. J Biol Chem (2010) 1.55
Cooperative assembly and misfolding of CFTR domains in vivo. Mol Biol Cell (2009) 1.31
Cystic fibrosis transmembrane regulator correctors and potentiators. Cold Spring Harb Perspect Med (2013) 1.20
ATP-independent CFTR channel gating and allosteric modulation by phosphorylation. Proc Natl Acad Sci U S A (2010) 1.19
DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers. Pulm Pharmacol Ther (2010) 1.16
Restoration of W1282X CFTR activity by enhanced expression. Am J Respir Cell Mol Biol (2007) 1.10
Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue. Am J Respir Cell Mol Biol (2009) 1.07
Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator. J Physiol (2009) 1.06
Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops. J Biol Chem (2011) 1.04
Potentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs. J Biol Chem (2010) 1.03
A unified view of cystic fibrosis transmembrane conductance regulator (CFTR) gating: combining the allosterism of a ligand-gated channel with the enzymatic activity of an ATP-binding cassette (ABC) transporter. J Biol Chem (2011) 1.03
Computational studies reveal phosphorylation-dependent changes in the unstructured R domain of CFTR. J Mol Biol (2008) 1.01
The CFTR ion channel: gating, regulation, and anion permeation. Cold Spring Harb Perspect Med (2013) 1.00
What's new in cystic fibrosis? From treating symptoms to correction of the basic defect. Eur J Pediatr (2008) 0.99
Partial correction of cystic fibrosis defects with PLGA nanoparticles encapsulating curcumin. Mol Pharm (2010) 0.99
Bcl-2 suppresses sarcoplasmic/endoplasmic reticulum Ca2+-ATPase expression in cystic fibrosis airways: role in oxidant-mediated cell death. Am J Respir Crit Care Med (2009) 0.94
Curcumin cross-links cystic fibrosis transmembrane conductance regulator (CFTR) polypeptides and potentiates CFTR channel activity by distinct mechanisms. J Biol Chem (2009) 0.93
Nonequilibrium gating of CFTR on an equilibrium theme. Physiology (Bethesda) (2012) 0.91
Allosteric coupling between the intracellular coupling helix 4 and regulatory sites of the first nucleotide-binding domain of CFTR. PLoS One (2013) 0.89
Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1. Br J Pharmacol (2011) 0.85
State-dependent regulation of cystic fibrosis transmembrane conductance regulator (CFTR) gating by a high affinity Fe3+ bridge between the regulatory domain and cytoplasmic loop 3. J Biol Chem (2010) 0.85
Curcumin affects cell survival and cell volume regulation in human renal and intestinal cells. Toxicology (2011) 0.83
Dynamics intrinsic to cystic fibrosis transmembrane conductance regulator function and stability. Cold Spring Harb Perspect Med (2013) 0.83
Molecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosis. Arch Biochem Biophys (2011) 0.83
Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps. J Biol Chem (2014) 0.82
Converting nonhydrolyzable nucleotides to strong cystic fibrosis transmembrane conductance regulator (CFTR) agonists by gain of function (GOF) mutations. J Biol Chem (2013) 0.82
Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations. Am J Respir Crit Care Med (2016) 0.82
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR): CLOSED AND OPEN STATE CHANNEL MODELS. J Biol Chem (2015) 0.81
The inhibition mechanism of non-phosphorylated Ser768 in the regulatory domain of cystic fibrosis transmembrane conductance regulator. J Biol Chem (2010) 0.80
Curcumin and genistein: the combined effects on disease-associated CFTR mutants and their clinical implications. Curr Pharm Des (2013) 0.80
Obligate coupling of CFTR pore opening to tight nucleotide-binding domain dimerization. Elife (2016) 0.78
Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives. Clin Pharmacol (2016) 0.77
Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators. PLoS One (2016) 0.77
Regulation of activation and processing of the cystic fibrosis transmembrane conductance regulator (CFTR) by a complex electrostatic interaction between the regulatory domain and cytoplasmic loop 3. J Biol Chem (2012) 0.76
Cross-linking of ΔF508-CFTR promotes its trafficking to the plasma membrane. Channels (Austin) (2010) 0.75
Cumulative association of five genetic variants with prostate cancer. N Engl J Med (2008) 15.34
The ageing systemic milieu negatively regulates neurogenesis and cognitive function. Nature (2011) 6.71
Glucose levels and risk of dementia. N Engl J Med (2013) 4.95
Two genome-wide association studies of aggressive prostate cancer implicate putative prostate tumor suppressor gene DAB2IP. J Natl Cancer Inst (2007) 4.85
Pathological correlates of dementia in a longitudinal, population-based sample of aging. Ann Neurol (2007) 4.47
Association between two unlinked loci at 8q24 and prostate cancer risk among European Americans. J Natl Cancer Inst (2007) 4.24
Reversal of persistent fibrosis in aging by targeting Nox4-Nrf2 redox imbalance. Sci Transl Med (2014) 3.23
Age and apolipoprotein E*4 allele effects on cerebrospinal fluid beta-amyloid 42 in adults with normal cognition. Arch Neurol (2006) 2.79
How to review a paper. Adv Physiol Educ (2003) 2.67
Sequence variants at 22q13 are associated with prostate cancer risk. Cancer Res (2009) 2.27
Sequence variants of toll-like receptor 4 are associated with prostate cancer risk: results from the CAncer Prostate in Sweden Study. Cancer Res (2004) 1.91
Effect of statins on Alzheimer's disease biomarkers in cerebrospinal fluid. J Alzheimers Dis (2006) 1.82
Inhibition of mechanosensitive signaling in myofibroblasts ameliorates experimental pulmonary fibrosis. J Clin Invest (2013) 1.81
A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA. Proc Natl Acad Sci U S A (2002) 1.78
Blood pressure and brain injury in older adults: findings from a community-based autopsy study. J Am Geriatr Soc (2009) 1.76
Association of prostate cancer risk variants with clinicopathologic characteristics of the disease. Clin Cancer Res (2008) 1.74
YKL-40: a novel prognostic fluid biomarker for preclinical Alzheimer's disease. Biol Psychiatry (2010) 1.59
Inherited genetic variant predisposes to aggressive but not indolent prostate cancer. Proc Natl Acad Sci U S A (2010) 1.59
SNPs associated with cerebrospinal fluid phospho-tau levels influence rate of decline in Alzheimer's disease. PLoS Genet (2010) 1.58
Sequence variants in Toll-like receptor gene cluster (TLR6-TLR1-TLR10) and prostate cancer risk. J Natl Cancer Inst (2005) 1.55
Prevalence of late amputations during the current conflicts in Afghanistan and Iraq. Mil Med (2010) 1.54
LL-37 enhances adaptive antitumor immune response in a murine model when genetically fused with M-CSFR (J6-1) DNA vaccine. Leuk Res (2005) 1.51
Multiple SNPs within and surrounding the apolipoprotein E gene influence cerebrospinal fluid apolipoprotein E protein levels. J Alzheimers Dis (2008) 1.40
Clinical significance of IL-18 gene over-expression in AML. Leuk Res (2002) 1.39
Acid-sensing ion channels in malignant gliomas. J Biol Chem (2003) 1.38
Identification and validation of novel cerebrospinal fluid biomarkers for staging early Alzheimer's disease. PLoS One (2011) 1.32
Association of a germ-line copy number variation at 2p24.3 and risk for aggressive prostate cancer. Cancer Res (2009) 1.29
Japanese encephalitis, Tibet, China. Emerg Infect Dis (2011) 1.26
Reversible silencing of CFTR chloride channels by glutathionylation. J Gen Physiol (2005) 1.22
Systematic replication study of reported genetic associations in prostate cancer: Strong support for genetic variation in the androgen pathway. Prostate (2006) 1.19
ATP-independent CFTR channel gating and allosteric modulation by phosphorylation. Proc Natl Acad Sci U S A (2010) 1.19
Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation. Proc Natl Acad Sci U S A (2004) 1.19
Loss of coronal correction following instrumentation removal in adolescent idiopathic scoliosis. Spine (Phila Pa 1976) (2006) 1.19
Association of 17 prostate cancer susceptibility loci with prostate cancer risk in Chinese men. Prostate (2010) 1.12
A polymorphism in the CDKN1B gene is associated with increased risk of hereditary prostate cancer. Cancer Res (2004) 1.11
Anti-flt1 peptide, a vascular endothelial growth factor receptor 1-specific hexapeptide, inhibits tumor growth and metastasis. Clin Cancer Res (2005) 1.11
Activating cystic fibrosis transmembrane conductance regulator channels with pore blocker analogs. J Biol Chem (2005) 1.11
Interactions of sequence variants in interleukin-1 receptor-associated kinase4 and the toll-like receptor 6-1-10 gene cluster increase prostate cancer risk. Cancer Epidemiol Biomarkers Prev (2006) 1.10
Socioeconomic status and hypertension: a meta-analysis. J Hypertens (2015) 1.09
Activity and expression of urokinase-type plasminogen activator and matrix metalloproteinases in human colorectal cancer. BMC Cancer (2006) 1.07
Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops. J Biol Chem (2011) 1.04
Neuropathology in the adult changes in thought study: a review. J Alzheimers Dis (2009) 1.02
IL-18 increases invasiveness of HL-60 myeloid leukemia cells: up-regulation of matrix metalloproteinases-9 (MMP-9) expression. Leuk Res (2004) 1.01
Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis. J Biol Chem (2005) 1.01
CFTR chloride channels are regulated by a SNAP-23/syntaxin 1A complex. Proc Natl Acad Sci U S A (2002) 1.01
The CFTR ion channel: gating, regulation, and anion permeation. Cold Spring Harb Perspect Med (2013) 1.00
Progesterone increases systemic and local uterine proportions of CD4+CD25+ Treg cells during midterm pregnancy in mice. Endocrinology (2010) 1.00
Mechanisms of CFTR regulation by syntaxin 1A and PKA. J Cell Sci (2002) 1.00
Discovery of 2-hydroxy-N,N-dimethyl-3-{2-[[(R)-1-(5- methylfuran-2-yl)propyl]amino]-3,4-dioxocyclobut-1-enylamino}benzamide (SCH 527123): a potent, orally bioavailable CXCR2/CXCR1 receptor antagonist. J Med Chem (2006) 0.99
CHFR protein expression predicts outcomes to taxane-based first line therapy in metastatic NSCLC. Clin Cancer Res (2013) 0.99
Histone modifications in senescence-associated resistance to apoptosis by oxidative stress. Redox Biol (2013) 0.98
A Luminex assay detects amyloid β oligomers in Alzheimer's disease cerebrospinal fluid. PLoS One (2013) 0.98
Straight-forward versus anatomic trajectory technique of thoracic pedicle screw fixation: a biomechanical analysis. Spine (Phila Pa 1976) (2003) 0.98
Class I HDACs are mediators of smoke carcinogen-induced stabilization of DNMT1 and serve as promising targets for chemoprevention of lung cancer. Cancer Prev Res (Phila) (2014) 0.97
Association between lifetime cigarette smoking and lewy body accumulation. Brain Pathol (2009) 0.95
Enhancing the power of genetic association studies through the use of silver standard cases derived from electronic medical records. PLoS One (2013) 0.94
Differential regulation of vimentin mRNA by 12-O-tetradecanoylphorbol 13-acetate and all-trans-retinoic acid correlates with motility of Hep 3B human hepatocellular carcinoma cells. Cancer Lett (2004) 0.94
Expression of P2X7 in human hematopoietic cell lines and leukemia patients. Leuk Res (2004) 0.93
Curcumin cross-links cystic fibrosis transmembrane conductance regulator (CFTR) polypeptides and potentiates CFTR channel activity by distinct mechanisms. J Biol Chem (2009) 0.93
Cystic fibrosis transmembrane conductance regulator trafficking is mediated by the COPI coat in epithelial cells. J Biol Chem (2007) 0.92
Gabexate mesilate inhibits colon cancer growth, invasion, and metastasis by reducing matrix metalloproteinases and angiogenesis. Clin Cancer Res (2004) 0.92
Modeling of pathological traits in Alzheimer's disease based on systemic extracellular signaling proteome. Mol Cell Proteomics (2011) 0.92
Protein-bound polysaccharide from Phellinus linteus inhibits tumor growth, invasion, and angiogenesis and alters Wnt/β-catenin in SW480 human colon cancer cells. BMC Cancer (2011) 0.92
Heterochromatin protein 1 promotes self-renewal and triggers regenerative proliferation in adult stem cells. J Cell Biol (2013) 0.92
The Effects of Velvet Antler of Deer on Cardiac Functions of Rats with Heart Failure following Myocardial Infarction. Evid Based Complement Alternat Med (2012) 0.92
Salivary tau species are potential biomarkers of Alzheimer's disease. J Alzheimers Dis (2011) 0.91
Vpr-host interactions during HIV-1 viral life cycle. J Neuroimmune Pharmacol (2011) 0.90
Delta-sarcoglycan is necessary for early heart and muscle development in zebrafish. Biochem Biophys Res Commun (2006) 0.88
Interactions between drugs and polymers influencing hot melt extrusion. J Pharm Pharmacol (2013) 0.88
Synthesis and structure-activity relationships of 3,4-diaminocyclobut-3-ene-1,2-dione CXCR2 antagonists. Bioorg Med Chem Lett (2006) 0.87
Lanosterol metabolic product(s) is involved in primordial folliculogenesis and establishment of primordial follicle pool in mouse fetal ovary. Mol Reprod Dev (2009) 0.87
Discovery of [7-(2,6-dichlorophenyl)-5-methylbenzo [1,2,4]triazin-3-yl]-[4-(2-pyrrolidin-1-ylethoxy)phenyl]amine--a potent, orally active Src kinase inhibitor with anti-tumor activity in preclinical assays. Bioorg Med Chem Lett (2006) 0.86
Comparative effect of orthosis design on functional performance. J Bone Joint Surg Am (2012) 0.86
Cubic phase nanoparticles for sustained release of ibuprofen: formulation, characterization, and enhanced bioavailability study. Int J Nanomedicine (2013) 0.86
Transfection of mEpo gene to intestinal epithelium in vivo mediated by oral delivery of chitosan-DNA nanoparticles. World J Gastroenterol (2004) 0.86
Detection of CXCR4 receptors on cell surface using a fluorescent metal nanoshell. J Biomed Opt (2011) 0.85
CFTR surface expression and chloride currents are decreased by inhibitors of N-WASP and actin polymerization. Biochim Biophys Acta (2006) 0.85
Differential diagnostic value of GPC3-CD34 combined staining in small liver nodules with diameter less than 3 cm. Am J Clin Pathol (2012) 0.85
The interaction between syntaxin 1A and cystic fibrosis transmembrane conductance regulator Cl- channels is mechanistically distinct from syntaxin 1A-SNARE interactions. J Biol Chem (2002) 0.85
In vitro and in vivo evaluation of ordered mesoporous silica as a novel adsorbent in liquisolid formulation. Int J Nanomedicine (2012) 0.85
PDCD5 negatively regulates autoimmunity by upregulating FOXP3(+) regulatory T cells and suppressing Th17 and Th1 responses. J Autoimmun (2013) 0.85
Argonaute-2 regulates the proliferation of adult stem cells in planarian. Cell Res (2011) 0.84
A comparison of limb-socket kinematics of bone-bridging and non-bone-bridging wartime transtibial amputations. J Bone Joint Surg Am (2012) 0.84
Foxp3+ regulatory T cells are associated with the natural history of chronic hepatitis B and poor prognosis of hepatocellular carcinoma. Liver Int (2011) 0.84
The characteristics of the synonymous codon usage in hepatitis B virus and the effects of host on the virus in codon usage pattern. Virol J (2011) 0.84
Pax4 paired domain mediates direct protein transduction into mammalian cells. Endocrinology (2007) 0.84
Expression and regulation of interleukin-23 subunits in human peripheral blood mononuclear cells and hematopoietic cell lines in response to various inducers. Cell Biol Int (2004) 0.84
Expression of LL-37/hCAP-18 gene in human leukemia cells. Leuk Res (2003) 0.84
Influence of heme oxygenase-1 gene transfer on the viability and function of rat islets in in vitro culture. World J Gastroenterol (2007) 0.84
Deployment after limb salvage for high-energy lower-extremity trauma. J Trauma Acute Care Surg (2012) 0.84
A novel type of PTD, common helix-loop-helix motif, could efficiently mediate protein transduction into mammalian cells. Biochem Biophys Res Commun (2006) 0.84
Direct observation to chemokine receptor 5 on T-lymphocyte cell surface using fluorescent metal nanoprobes. Biochem Biophys Res Commun (2010) 0.84
Retinoic acid derived from the fetal ovary initiates meiosis in mouse germ cells. J Cell Physiol (2013) 0.83
Quantal release of free radicals during exocytosis of phagosomes. Nat Cell Biol (2002) 0.83
Transmembrane protein 208: a novel ER-localized protein that regulates autophagy and ER stress. PLoS One (2013) 0.83
Human immunodeficiency virus type 1 Vpr induces cell cycle G2 arrest through Srk1/MK2-mediated phosphorylation of Cdc25. J Virol (2007) 0.83