Standout Papers
Citation Impact
Citing Papers
Histone demethylase KDM6A directly senses oxygen to control chromatin and cell fate
2019 StandoutScienceNobel
Targetable Signaling Pathway Mutations Are Associated with Malignant Phenotype in IDH -Mutant Gliomas
2014
De novo design of transmembrane β barrels
2021 StandoutScienceNobel
Conformational Switch of Syntaxin-1 Controls Synaptic Vesicle Fusion
2008 StandoutScienceNobel
Gating the Selectivity Filter in ClC Chloride Channels
2003 StandoutScienceNobel
Failure of cholinergic stimulation to induce a secretory response from the rectal mucosa in cystic fibrosis.
1991
Suppression of Hypoxia-Inducible Factor 2α Restores p53 Activity via Hdm2 and Reverses Chemoresistance of Renal Carcinoma Cells
2009
On-target efficacy of a HIF-2α antagonist in preclinical kidney cancer models
2016 StandoutNatureNobel
Purification and Characterization of Recombinant Cystic Fibrosis Transmembrane Conductance Regulator from Chinese Hamster Ovary and Insect Cells
1995
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
1992
Extensive posttranscriptional deletion of the coding sequences for part of nucleotide-binding fold 1 in respiratory epithelial mRNA transcripts of the cystic fibrosis transmembrane conductance regulator gene is not associated with the clinical manifestations of cystic fibrosis.
1992
Defective acidification of intracellular organelles in cystic fibrosis
1991 Nature
Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
1991 Nature
Long-term uncoupling of chloride secretion from intracellular calcium levels by lns(3,4,5,6)P4
1994 StandoutNatureNobel
Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer
1990 Standout
Gene therapy for cystic fibrosis: challenges and future directions.
1995
Generation and characterization of a ΔF508 cystic fibrosis mouse model
1995 StandoutNobel
A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein
1990 Nature
Localization and Suppression of a Kinetic Defect in Cystic Fibrosis Transmembrane Conductance Regulator Folding
1997
Effects of the ?F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR
1993
Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis
1991
Expression and characterization of the cystic fibrosis transmembrane conductance regulator
1990 Nature
Omission of exon 12 in cystic fibrosis transmembrane conductance regulator (CFTR) gene transcripts
1992
Chloride channels, Golgi pH and cystic fibrosis
1992
Genetic mapping of a gene causing hypertension in the stroke-prone spontaneously hypertensive rat
1991
Localization of the human multiple drug resistance gene, MDR1, to 7q21.1
1987
Activation of chloride channels in normal and cystic fibrosis airway epithelial cells by multifunctional calcium/calmodulin-dependent protein kinase
1991 Nature
Activation by Extracellular Nucleotides of Chloride Secretion in the Airway Epithelia of Patients with Cystic Fibrosis
1991
Rapid gating and anion permeability of an intracellular aquaporin
1999 StandoutNatureNobel
Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis
2002
Structural model of ATP-binding proteing associated with cystic fibrosis, multidrug resistance and bacterial transport
1990 Nature
In vivo transfer of the human cystic fibrosis transmembrane conductance regulator gene to the airway epithelium
1992
Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study
2003
Defective regulation of outwardly rectifying Cl− channels by protein kinase A corrected by insertion of CFTR
1992 Nature
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
1992 Nature
Nucleoside triphosphates are required to open the CFTR chloride channel
1991
Functional Repair of CFTR by CRISPR/Cas9 in Intestinal Stem Cell Organoids of Cystic Fibrosis Patients
2013
Structure of the recA protein–ADP complex
1992 StandoutNatureNobel
Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium
1991
A frame-shift mutation in the cystic fibrosis gene
1990 Nature
Cloning and expression of an inwardly rectifying ATP-regulated potassium channel
1993 Nature
In vivo evidence of altered chloride but not potassium secretion in cystic fibrosis rectal mucosa
1991
Production of a severe cystic fibrosis mutation in mice by gene targeting
1993 StandoutNobel
Small-intestinal abnormalities in cystic fibrosis patients
1991
New structural motif for ligand-gated ion channels defined by an ionotropic ATP receptor
1994 StandoutNatureNobel
Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA
1993
Identification of protein coding regions by database similarity search
1993
Disruption of the cystic fibrosis transmembrane conductance regulator gene in embryonic stem cells by gene targeting
1992 StandoutNobel
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
1993
Expression of Multidrug Resistance Gene in Human Cancers
1989
Cystic Fibrosis
2005
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
2011 Standout
Electrostatic tuning of Mg2+ affinity in an inward-rectifier K+channel
1994 StandoutNatureNobel
Inactivation of the murine cftr gene abolishes cAMP-mediated but not Ca2+-mediated secretagogue-induced volume decrease in small-intestinal crypts
1993 StandoutNobel
Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms
1997 Nature
Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland
1992
The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR
1993
Successful targeting of the mouse cystic fibrosis transmembrane conductance regulator gene in embryonal stem cells
1992
Strategies for Correcting the AF508 CFTR Protein-Folding Defect
1997
Epithelial cancer in Fanconi anemia complementation group D2 (Fancd2) knockout mice
2003
Salmonella typhi uses CFTR to enter intestinal epithelial cells
1998 StandoutNatureNobel
Expression of the human cystic fibrosis transmembrane conductance regulator gene in the mouse lung afterin vivointratracheal plasmid-mediated gene transfer
1992
Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization.
1994
Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator (CFTR) gene
1991
Elevated expression of Annexin II (Lipocortin II, p36) in a multidrug resistant small cell lung cancer cell line
1992
In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator
1991 Nature
The Human ATP-Binding Cassette (ABC) Transporter Superfamily
2001
CFTR expression is regulated during both the cycle of the seminiferous epithelium and the oestrous cycle of rodents
1993
Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.
1991
Overcoming Hysteresis to Attain Reversible Equilibrium Folding for Outer Membrane Phospholipase A in Phospholipid Bilayers
2011
Quantitative expression patterns of multidrug‐resistance P‐glycoprotein (MDR1) and differentially spliced cystic‐fibrosis transmembrane‐conductance regulator mRNA transcripts in human epithelia
1992
Male–female differences in fertility and blood pressure in ACE-deficient mice
1995 StandoutNatureNobel
Activation of ion transport pathways by changes in cell volume
1991
Cystic fibrosis: molecular biology and therapeutic implications
1992 StandoutScience
A functional CFTR assay using primary cystic fibrosis intestinal organoids
2013
Therapy of CF-Patients with Amitriptyline and Placebo - a Randomised, Double-Blind, Placebo-Controlled Phase IIb Multicenter, Cohort-Study
2013 Standout
Surrogate Wnt agonists that phenocopy canonical Wnt and β-catenin signalling
2017 StandoutNatureNobel
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
1990 Standout
Submucosal glands are the predominant site of CFTR expression in the human bronchus
1992
DNA-PKcs has KU-dependent function in rRNA processing and haematopoiesis
2020 StandoutNatureNobel
Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance
1991
Cystic fibrosis in the mouse by targeted insertional mutagenesis
1992 Nature
Non–invasive liposome–mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice
1993
Basic local alignment search tool
1990 Standout
Degradation of CFTR by the ubiquitin-proteasome pathway
1995 Standout
X-ray structure of a ClC chloride channel at 3.0 Å reveals the molecular basis of anion selectivity
2002 StandoutNatureNobel
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
1990 Nature
Molecular cloning and sequence analysis of the murine cDNA for the cystic fibrosis transmembrane conductance regulator
1991
Disruption of c-mos causes parthenogenetic development of unfertilized mouse eggs
1994 StandoutNatureNobel
A mouse model for the delta F508 allele of cystic fibrosis.
1995 StandoutNobel
Mutations in the Cystic Fibrosis Gene in Patients with Congenital Absence of the Vas Deferens
1995 Standout
The Endoplasmic Reticulum as a Site of Protein Degradation
1993
The spectrum of cystic fibrosis mutations
1992
Liposome-mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis
1995 Standout
A Novel Mutation in the Cystic Fibrosis Gene in Patients with Pulmonary Disease but Normal Sweat Chloride Concentrations
1994
Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy
1993 StandoutNatureNobel
Mutation Analysis for Heterozygote Detection and the Prenatal Diagnosis of Cystic Fibrosis
1990
Cloning the mouse homolog of the human cystic fibrosis transmembrane conductance regulator gene
1991
Multiple mutations in highly conserved residues are found in mildly affected cystic fibrosis patients
1990
Gapped BLAST and PSI-BLAST: a new generation of protein database search programs
1997 Standout
Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type Cftr gene expression
1994
The Cystic Fibrosis Transmembrane Conductance Regulator Gene
1995
Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis
2003 Standout
Differential Localization of the Cystic Fibrosis Transmembrane Conductance Regulator in Normal and Cystic Fibrosis Airway Epithelium
1992
Overexpression and amplification of five genes in a multidrug-resistant Chinese hamster ovary cell line.
1986
Antisense oligodeoxynucleotides to the cystic fibrosis transmembrane conductance regulator inhibit cAMP-activated but not calcium-activated chloride currents.
1992
An Animal Model for Cystic Fibrosis Made by Gene Targeting
1992 StandoutScienceNobel
Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen).
1994
Chloride secretory response to extracellular ATP in human normal and cystic fibrosis nasal epithelia
1992
Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes
1991 Science
Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping
1989 StandoutScience
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.
1993
Kap104p: A Karyopherin Involved in the Nuclear Transport of Messenger RNA Binding Proteins
1996 StandoutScienceNobel
Is congenital bilateral absence of vas deferens a primary form of cystic fibrosis? Analyses of the CFTR gene in 67 patients.
1995
Voltage-dependent block of the cystic fibrosis transmembrane conductance regulator Cl- channel by two closely related arylaminobenzoates.
1993
The amplified H circle of methotrexate-resistant leishmania tarentolae contains a novel P-glycoprotein gene.
1990
Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia.
1992
Exon 9 of the CFTR gene: splice site haplotypes and cystic fibrosis mutations
1994
Effect of ATP-sensitive K+ channel regulators on cystic fibrosis transmembrane conductance regulator chloride currents.
1992
Stable in vivo expression of the cystic fibrosis transmembrane conductance regulator with an adeno-associated virus vector.
1993
Nasal potential difference: a clinical diagnostic test for cystic fibrosis
1990
Expression of the cystic fibrosis transmembrane conductance regulator gene in the respiratory tract of normal individuals and individuals with cystic fibrosis.
1991
Vinblastine photoaffinity labeling of a high molecular weight surface membrane glycoprotein specific for multidrug-resistant cells.
1986
CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2006-2010
2013 Standout
Defective Epithelial Chloride Transport in a Gene-Targeted Mouse Model of Cystic Fibrosis
1992 StandoutScienceNobel
The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells.
1993
Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity
1991 Science
Enhanced Myocardial Function in Transgenic Mice Overexpressing the β 2 -Adrenergic Receptor
1994 StandoutScienceNobel
HIF2α inhibition promotes p53 pathway activity, tumor cell death, and radiation responses
2009
Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes
1990 Nature
The new frontier of genome engineering with CRISPR-Cas9
2014 StandoutScienceNobel
The future of cloning
1999 StandoutNatureNobel
Potent and specific inhibition of mammalian histone deacetylase both in vivo and in vitro by trichostatin A.
1990 Standout
Forskolin Stimulation of Water and Cation Permeability in Aquaporin1 Water Channels
1996 Science
Multidrug resistance in a human small cell lung cancer cell line selected in adriamycin.
1987
Mapping of DNA markers linked to the cystic fibrosis locus on the long arm of chromosome 7.
1987
DAF-16 Target Genes That Control C. elegans Life-Span and Metabolism
2003 StandoutScienceNobel
Identification of mutations in regions corresponding to the two putative nucleotide (ATP)-binding folds of the cystic fibrosis gene.
1990
De novo design of protein homo-oligomers with modular hydrogen-bond network–mediated specificity
2016 StandoutScienceNobel
Homology between P-glycoprotein and a bacterial haemolysin transport protein suggests a model for multidrug resistance
1986 Nature
Location of Crossovers during Gene Targeting with Insertion and Replacement Vectors
1993 StandoutNobel
Molecular Structure and Physiological Function of Chloride Channels
2002
Screening and identification of a gene, PSE-1, that affects protein secretion in Saccharomyces cerevisiae
1992
Human Multidrug-Resistant Cell Lines: Increased mdr 1 Expression Can Precede Gene Amplification
1986 Science
Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.
1994
Abnormal regulation of ion channels in cystic fibrosis epithelia
1990
Chloride secretion in the trachea of null cystic fibrosis mice: the effects of transfection with pTrial10‐CFTR2.
1997 StandoutNobel
Detection of P-glycoprotein in multidrug-resistant cell lines by monoclonal antibodies
1985 Nature
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by negative charge in the R domain.
1993
Cystic ibrosis: Molecular Biology and Therapeutic Implications
1992 Science
Isolation and expression of a complementary DNA that confers multidrug resistance
1986 Nature
Multidrug Resistance of DNA-Mediated Transformants is Linked to Transfer of the Human mdr1 Gene
1986
Mechanism of cholesterol efflux from cells
1980
Variable deletion of exon 9 coding sequences in cystic fibrosis transmembrane conductance regulator gene mRNA transcripts in normal bronchial epithelium.
1991
Identification of the Cystic Fibrosis Gene: Genetic Analysis
1989 StandoutScience
Methods for assessing the statistical significance of molecular sequence features by using general scoring schemes.
1990
Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro.
1994
Amino acid substitution matrices from an information theoretic perspective
1991
Amplification and Expression of Genes Associated with Multidrug Resistance in Mammalian Cells
1986 Science
Physical localization of two DNA markers closely linked to the cystic fibrosis locus by pulsed-field gel electrophoresis.
1989
Genetic and biochemical characterization of multidrug resistance
1985
Isolation of human mdr DNA sequences amplified in multidrug-resistant KB carcinoma cells.
1986
Correlation between Genotype and Phenotype in Patients with Cystic Fibrosis
1993
Location of crossovers during gene targeting with insertion and replacement vectors.
1993
Cystic fibrosis: a disease in electrolyte transport
1990
Internal duplication and homology with bacterial transport proteins in the mdr1 (P-glycoprotein) gene from multidrug-resistant human cells
1986 Standout
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.
1994
Biogenesis of γ-secretase early in the secretory pathway
2007 StandoutNobel
Cystic Fibrosis Heterozygote Resistance to Cholera Toxin in the Cystic Fibrosis Mouse Model
1994 Science
Independent Pathways for Secretion of Cholesterol and Apolipoprotein E by Macrophages
1983 StandoutScienceNobel
Immunocytochemical localization of the cystic fibrosis gene product CFTR.
1991
Integrating Genetic Approaches into the Discovery of Anticancer Drugs
1997 StandoutScienceNobel
Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2.
1991
Structure of an mdr-like gene from Arabidopsis thaliana. Evolutionary implications.
1992
Impaired cell volume regulation in intestinal crypt epithelia of cystic fibrosis mice.
1995 StandoutNobel
Localization of multidrug resistance-associated DNA sequences to human chromosome 7
1986
Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis
2002
Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia.
1991
Genetic determination of exocrine pancreatic function in cystic fibrosis.
1992
Effect of Deleting the R Domain on CFTR-Generated Chloride Channels
1991 Science
Production and characterisation of monoclonal and polyclonal antibodies to different regions of the cystic fibrosis transmembrane conductance regulator (cftr): Detection of immunologically related proteins
1995
Overexpression of a Transporter Gene in a Multidrug-Resistant Human Lung Cancer Cell Line
1992 StandoutScience
Differential acidic pH sensitivity of delta F508 CFTR Cl- channel activity in lipid bilayers
1994
Works of Noa Alon being referenced
Monoallelic Expression Determines Oncogenic Progression and Outcome in Benign and Malignant Brain Tumors
2011
BRAF-KIAA1549 Fusion Predicts Better Clinical Outcome in Pediatric Low-Grade Astrocytoma
2011
Expression of p53 in renal carcinoma cells is independent of pVHL
2005
Isolation of a cDNA Representing the Fanconi Anemia Complementation Group E Gene
2000
The Fanconi anaemia gene FANCF encodes a novel protein with homology to ROM
2000
The CF Gene Product as a Member of a Membrane Transporter (TM6-NBF) Super Family
1991
Expression cloning of a cDNA for the major Fanconi anaemia gene, FAA
1996
The Fanconi anaemia group G gene FANCG is identical with XRCC9
1998
Plasma membrane lipids of human diploid fibroblasts from normal individuals and patients with cystic fibrosis
1979
Amplification of P-glycoprotein genes in multidrug-resistant mammalian cell lines
1985 Nature
Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites
1993
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
1989 StandoutScience