Standout Papers
Citation Impact
Citing Papers
DNA capture by a CRISPR-Cas9–guided adenine base editor
2020 StandoutScienceNobel
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
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
A Preliminary Study of Aerosolized Recombinant Human Deoxyribonuclease I in the Treatment of Cystic Fibrosis
1992
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
Human ADAR1 Prevents Endogenous RNA from Triggering Translational Shutdown
2018 StandoutNobel
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
MHC class II region encoding proteins related to the muKidrug resistance family of transmembrane transporters
1990 Nature
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
A protein-conducting channel in the endoplasmic reticulum
1991 StandoutNobel
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
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
Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel
1991
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
Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel.
1991
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
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
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
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
A Multigene Family Encoding a Diverse Array of Putative Pheromone Receptors in Mammals
1997 StandoutNobel
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
Long-Term Expansion of Functional Mouse and Human Hepatocytes as 3D Organoids
2018 StandoutNobel
Cystic fibrosis: molecular biology and therapeutic implications
1992 StandoutScience
Structures of human ADAR2 bound to dsRNA reveal base-flipping mechanism and basis for site selectivity
2016
SWELL1, a Plasma Membrane Protein, Is an Essential Component of Volume-Regulated Anion Channel
2014 StandoutNobel
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
Receptors and G proteins as primary components of transmembrane signal transduction
1995
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
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
CFTR: Development of high- affinity antibodies and localization in sweat gland
1991
RADAR: a rigorously annotated database of A-to-I RNA editing
2013
Non–invasive liposome–mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice
1993
Principles Governing A-to-I RNA Editing in the Breast Cancer Transcriptome
2015
Basic local alignment search tool
1990 Standout
The CF Gene Product as a Member of a Membrane Transporter (TM6-NBF) Super Family
1991
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 Relation between Genotype and Phenotype in Cystic Fibrosis — Analysis of the Most Common Mutation (ΔF508)
1990
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
Structure and function of haemolysin B, P‐glycoprotein and other members of a novel family of membrane translocators
1990
Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy
1993 StandoutNatureNobel
Phosphorylation-regulated CI− channel in CHO cells stably expressing the cystic fibrosis gene
1991 Nature
Mutation Analysis for Heterozygote Detection and the Prenatal Diagnosis of Cystic Fibrosis
1990
Elevated RNA Editing Activity Is a Major Contributor to Transcriptomic Diversity in Tumors
2015
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
Functional and genetic deconstruction of the cellular origin in liver cancer
2015
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
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
Analysis of four diverse population groups indicates that a subset of cystic fibrosis mutations occur in common among Caucasians.
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
Generation of cAMP-Activated Chloride Currents by Expression of CFTR
1991 Science
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
Crystal Structure of Human BPI and Two Bound Phospholipids at 2.4 Angstrom Resolution
1997 Science
Defective Epithelial Chloride Transport in a Gene-Targeted Mouse Model of Cystic Fibrosis
1992 StandoutScienceNobel
Biochemical characterization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis epithelial cells.
1992
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
GENETICS OF NON-INSULIN-DEPENDENT (TYPE-II) DIABETES MELLITUS
1996
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
Cystic Fibrosis Gene Mutation in Two Sisters with Mild Disease and Normal Sweat Electrolyte Levels
1991
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
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
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
Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum.
1990
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
Cl- channel activity in Xenopus oocytes expressing the cystic fibrosis gene.
1991
Positional cloning: Let's not call it reverse anymore
1992
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
The family of G‐protein‐coupled receptors
1995
Amino acid substitution matrices from an information theoretic perspective
1991
Physical localization of two DNA markers closely linked to the cystic fibrosis locus by pulsed-field gel electrophoresis.
1989
CFTR protein expression in primary and cultured epithelia.
1992
Correlation between Genotype and Phenotype in Patients with Cystic Fibrosis
1993
Location of crossovers during gene targeting with insertion and replacement vectors.
1993
daf-2 , an Insulin Receptor-Like Gene That Regulates Longevity and Diapause in Caenorhabditis elegans
1997 StandoutScienceNobel
Cystic fibrosis: a disease in electrolyte transport
1990
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
Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line.
1992
Genetic analysis of an MDR-like export system: the secretion of colicin V.
1990
Immunocytochemical localization of the cystic fibrosis gene product CFTR.
1991
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
Phylogenetic Perspectives in Innate Immunity
1999 StandoutScienceNobel
Impaired cell volume regulation in intestinal crypt epithelia of cystic fibrosis mice.
1995 StandoutNobel
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
Transduction of Receptor Signals by ß-Arrestins
2005 StandoutScienceNobel
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
cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator.
1991
Works of Si Lok being referenced
The human glucagon receptor encoding gene: structure, cDNA sequence and chromosomal localization
1994
Functional expression of human and mouse plasma phospholipid transfer protein: effect of recombinant and plasma PLTP on HDL subspecies
1995
Recoding RNA editing of AZIN1 predisposes to hepatocellular carcinoma
2013
Expression cloning and signaling properties of the rat glucagon receptor
1993 Science
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
1989 StandoutScience