Published in Cell Mol Life Sci on August 26, 2009
CFTR: folding, misfolding and correcting the ΔF508 conformational defect. Trends Mol Med (2011) 1.99
Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function. Cell (2012) 1.73
Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR. J Mol Biol (2010) 1.31
Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation. J Gen Physiol (2010) 1.26
Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein. FASEB J (2012) 1.17
The primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domain. PLoS One (2010) 1.17
Pharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platform. Mol Med (2012) 1.15
Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions. Proc Natl Acad Sci U S A (2013) 1.06
Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem (2010) 1.03
The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate. J Biol Chem (2011) 1.02
The CFTR ion channel: gating, regulation, and anion permeation. Cold Spring Harb Perspect Med (2013) 1.00
Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Gen Physiol (2011) 0.98
Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant. Pflugers Arch (2011) 0.96
Locating a plausible binding site for an open-channel blocker, GlyH-101, in the pore of the cystic fibrosis transmembrane conductance regulator. Mol Pharmacol (2012) 0.96
Cystic fibrosis transmembrane conductance regulator: a molecular model defines the architecture of the anion conduction path and locates a "bottleneck" in the pore. Biochemistry (2012) 0.94
A chaperone trap contributes to the onset of cystic fibrosis. PLoS One (2012) 0.93
Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant. J Biol Chem (2011) 0.93
Modeling the conformational changes underlying channel opening in CFTR. PLoS One (2013) 0.92
Two salt bridges differentially contribute to the maintenance of cystic fibrosis transmembrane conductance regulator (CFTR) channel function. J Biol Chem (2013) 0.90
Functional Rescue of F508del-CFTR Using Small Molecule Correctors. Front Pharmacol (2012) 0.90
Differential contribution of TM6 and TM12 to the pore of CFTR identified by three sulfonylurea-based blockers. Pflugers Arch (2011) 0.90
Alternating access to the transmembrane domain of the ATP-binding cassette protein cystic fibrosis transmembrane conductance regulator (ABCC7). J Biol Chem (2012) 0.89
Conformational changes relevant to channel activity and folding within the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator. J Biol Chem (2012) 0.89
The human transporter associated with antigen processing: molecular models to describe peptide binding competent states. J Biol Chem (2012) 0.89
Cysteine scanning of CFTR's first transmembrane segment reveals its plausible roles in gating and permeation. Biophys J (2013) 0.88
Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis. Acta Pharmacol Sin (2011) 0.88
An electrostatic interaction at the tetrahelix bundle promotes phosphorylation-dependent cystic fibrosis transmembrane conductance regulator (CFTR) channel opening. J Biol Chem (2014) 0.87
Cystic fibrosis transmembrane conductance regulator (ABCC7) structure. Cold Spring Harb Perspect Med (2013) 0.87
Structural changes of CFTR R region upon phosphorylation: a plastic platform for intramolecular and intermolecular interactions. FEBS J (2013) 0.86
Three charged amino acids in extracellular loop 1 are involved in maintaining the outer pore architecture of CFTR. J Gen Physiol (2014) 0.86
The role of the cytosolic HSP70 chaperone system in diseases caused by misfolding and aberrant trafficking of ion channels. Dis Model Mech (2014) 0.86
Phenotype-optimized sequence ensembles substantially improve prediction of disease-causing mutation in cystic fibrosis. Hum Mutat (2012) 0.85
Dynamics intrinsic to cystic fibrosis transmembrane conductance regulator function and stability. Cold Spring Harb Perspect Med (2013) 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
Restoration of NBD1 thermal stability is necessary and sufficient to correct ∆F508 CFTR folding and assembly. J Mol Biol (2014) 0.82
Relative contribution of different transmembrane segments to the CFTR chloride channel pore. Pflugers Arch (2013) 0.82
C terminus of nucleotide binding domain 1 contains critical features for cystic fibrosis transmembrane conductance regulator trafficking and activation. J Biol Chem (2010) 0.81
CFTR inhibitors. Curr Pharm Des (2013) 0.81
Metal bridges illuminate transmembrane domain movements during gating of the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem (2014) 0.81
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR): CLOSED AND OPEN STATE CHANNEL MODELS. J Biol Chem (2015) 0.81
Role of the juxtamembrane region of cytoplasmic loop 3 in the gating and conductance of the cystic fibrosis transmembrane conductance regulator chloride channel. Biochemistry (2012) 0.80
Modulation of cystic fibrosis transmembrane conductance regulator (CFTR) activity and genistein binding by cytosolic pH. J Biol Chem (2010) 0.80
CFTR is a mechanosensitive anion channel: a real stretch? Cellscience (2010) 0.79
Integrated analysis of residue coevolution and protein structure in ABC transporters. PLoS One (2012) 0.78
Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating. J Physiol (2015) 0.78
Cystic fibrosis lung environment and Pseudomonas aeruginosa infection. BMC Pulm Med (2016) 0.77
ATP and AMP mutually influence their interaction with the ATP-binding cassette (ABC) adenylate kinase cystic fibrosis transmembrane conductance regulator (CFTR) at separate binding sites. J Biol Chem (2013) 0.77
Functional Architecture of the Cytoplasmic Entrance to the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Pore. J Biol Chem (2015) 0.77
Development of CFTR Structure. Front Pharmacol (2012) 0.77
Introduction to section IV: biophysical methods to approach CFTR structure. Methods Mol Biol (2011) 0.76
The cystic fibrosis transmembrane conductance regulator (CFTR) and its stability. Cell Mol Life Sci (2016) 0.75
Decoding F508del misfolding in cystic fibrosis. Biomolecules (2014) 0.75
Genome-wide Membrane Protein Structure Prediction. Curr Genomics (2013) 0.75
Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis. Ann Thorac Med (2017) 0.75
Biophysical Approaches Facilitate Computational Drug Discovery for ATP-Binding Cassette Proteins. Int J Med Chem (2017) 0.75
Transmembrane helical interactions in the CFTR channel pore. PLoS Comput Biol (2017) 0.75
Small-angle X-ray scattering study of the ATP modulation of the structural features of the nucleotide binding domains of the CFTR in solution. Eur Biophys J (2011) 0.75
Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3). Cell (2003) 4.94
NEDD1-dependent recruitment of the gamma-tubulin ring complex to the centrosome is necessary for centriole duplication and spindle assembly. J Cell Biol (2006) 2.38
Molecular basis of the spectral expression of CIAS1 mutations associated with phagocytic cell-mediated autoinflammatory disorders CINCA/NOMID, MWS, and FCU. Blood (2003) 2.11
The Hedgehog-inducible ubiquitin ligase subunit WSB-1 modulates thyroid hormone activation and PTHrP secretion in the developing growth plate. Nat Cell Biol (2005) 1.78
Toxoplasma sortilin-like receptor regulates protein transport and is essential for apical secretory organelle biogenesis and host infection. Cell Host Microbe (2012) 1.78
The Tudor tandem of 53BP1: a new structural motif involved in DNA and RG-rich peptide binding. Structure (2004) 1.51
Inherited STING-activating mutation underlies a familial inflammatory syndrome with lupus-like manifestations. J Clin Invest (2014) 1.50
Multiple pathways inhibit NHEJ at telomeres. Genes Dev (2008) 1.40
The delta subunit of retinal rod cGMP phosphodiesterase regulates the membrane association of Ras and Rap GTPases. J Biol Chem (2002) 1.38
Characterization of a Type III secretion substrate specificity switch (T3S4) domain in YscP from Yersinia enterocolitica. Mol Microbiol (2005) 1.25
The discovery of SycO highlights a new function for type III secretion effector chaperones. EMBO J (2006) 1.23
Human RTEL1 deficiency causes Hoyeraal-Hreidarsson syndrome with short telomeres and genome instability. Hum Mol Genet (2013) 1.23
The A14-A43 heterodimer subunit in yeast RNA pol I and their relationship to Rpb4-Rpb7 pol II subunits. Proc Natl Acad Sci U S A (2002) 1.20
Structural characterization of filaments formed by human Xrcc4-Cernunnos/XLF complex involved in nonhomologous DNA end-joining. Proc Natl Acad Sci U S A (2011) 1.18
Ubiquitination-induced conformational change within the deiodinase dimer is a switch regulating enzyme activity. Mol Cell Biol (2007) 1.17
The metallo-beta-lactamase/beta-CASP domain of Artemis constitutes the catalytic core for V(D)J recombination. J Exp Med (2004) 1.16
Atypical structure and phylogenomic evolution of the new eutherian oocyte- and embryo-expressed KHDC1/DPPA5/ECAT1/OOEP gene family. Genomics (2007) 1.16
The munc13-4-rab27 complex is specifically required for tethering secretory lysosomes at the plasma membrane. Blood (2011) 1.14
An Rpb4/Rpb7-like complex in yeast RNA polymerase III contains the orthologue of mammalian CGRP-RCP. Mol Cell Biol (2003) 1.14
The multifunctional protein PEA-15 is involved in the control of apoptosis and cell cycle in astrocytes. Biochem Pharmacol (2003) 1.13
Function of Apollo (SNM1B) at telomere highlighted by a splice variant identified in a patient with Hoyeraal-Hreidarsson syndrome. Proc Natl Acad Sci U S A (2010) 1.12
Delineation of the Xrcc4-interacting region in the globular head domain of cernunnos/XLF. J Biol Chem (2010) 1.09
Human mismatch repair protein MSH6 contains a PWWP domain that targets double stranded DNA. Biochemistry (2008) 1.09
Self-assembled lamellar complexes of siRNA with lipidic aminoglycoside derivatives promote efficient siRNA delivery and interference. Proc Natl Acad Sci U S A (2007) 1.08
Identification of the hydrophobic strand in the A-B loop of leptin as major binding site III: implications for large-scale preparation of potent recombinant human and ovine leptin antagonists. Biochem J (2005) 1.07
The DFNA5 gene, responsible for hearing loss and involved in cancer, encodes a novel apoptosis-inducing protein. Eur J Hum Genet (2011) 1.03
Whole-exome sequencing identifies Coronin-1A deficiency in 3 siblings with immunodeficiency and EBV-associated B-cell lymphoproliferation. J Allergy Clin Immunol (2013) 1.00
The thyroid hormone-inactivating deiodinase functions as a homodimer. Mol Endocrinol (2008) 0.98
Phylogenetic analysis and identification of pseudogenes reveal a progressive loss of zona pellucida genes during evolution of vertebrates. Biol Reprod (2007) 0.98
Bovine leukemia virus SU protein interacts with zinc, and mutations within two interacting regions differently affect viral fusion and infectivity in vivo. J Virol (2002) 0.98
A new rubisco-like protein coexists with a photosynthetic rubisco in the planktonic cyanobacteria Microcystis. J Biol Chem (2006) 0.98
A generalized analysis of hydrophobic and loop clusters within globular protein sequences. BMC Struct Biol (2007) 0.96
The yeast Sar exchange factor Sec12, and its higher organism orthologs, fold as beta-propellers. FEBS Lett (2002) 0.95
Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics. Cell Mol Life Sci (2014) 0.95
Identification of a new expanding family of genes characterized by atypical LRR domains. Localization of a cluster preferentially expressed in oocyte. FEBS Lett (2003) 0.95
In silico identification and structural features of six new genes similar to MATER specifically expressed in the oocyte. Biochem Biophys Res Commun (2004) 0.95
Progress in cationic lipid-mediated gene transfection: a series of bio-inspired lipids as an example. Curr Gene Ther (2008) 0.94
A novel Toxoplasma gondii nuclear factor TgNF3 is a dynamic chromatin-associated component, modulator of nucleolar architecture and parasite virulence. PLoS Pathog (2011) 0.93
The C-terminal domain of Cernunnos/XLF is dispensable for DNA repair in vivo. Mol Cell Biol (2008) 0.93
Primary T-cell immunodeficiency with immunodysregulation caused by autosomal recessive LCK deficiency. J Allergy Clin Immunol (2012) 0.93
GPR50 is the mammalian ortholog of Mel1c: evidence of rapid evolution in mammals. BMC Evol Biol (2008) 0.93
Identification, characterization and metagenome analysis of oocyte-specific genes organized in clusters in the mouse genome. BMC Genomics (2005) 0.92
Nonionic amphiphilic block copolymers promote gene transfer to the lung. Hum Gene Ther (2005) 0.92
End-joining inhibition at telomeres requires the translocase and polySUMO-dependent ubiquitin ligase Uls1. EMBO J (2013) 0.92
Preparation of leptin antagonists by site-directed mutagenesis of human, ovine, rat, and mouse leptin's site III: implications on blocking undesired leptin action in vivo. Ann N Y Acad Sci (2006) 0.90
Structural and functional characterization of the TgDRE multidomain protein, a DNA repair enzyme from Toxoplasma gondii. Biochemistry (2006) 0.90
The checkpoint Saccharomyces cerevisiae Rad9 protein contains a tandem tudor domain that recognizes DNA. Nucleic Acids Res (2007) 0.90
D53 is a novel endosomal SNARE-binding protein that enhances interaction of syntaxin 1 with the synaptobrevin 2 complex in vitro. Biochem J (2003) 0.89
Severe and progressive encephalitis as a presenting manifestation of a novel missense perforin mutation and impaired cytolytic activity. Blood (2004) 0.89
Novel cationic lipids incorporating an acid-sensitive acylhydrazone linker: synthesis and transfection properties. J Med Chem (2004) 0.89
A histidine in the beta-CASP domain of Artemis is critical for its full in vitro and in vivo functions. DNA Repair (Amst) (2008) 0.88
ATP hydrolysis is essential for the function of the Uup ATP-binding cassette ATPase in precise excision of transposons. J Biol Chem (2006) 0.87
P3 peptide, a truncated form of A beta devoid of synaptotoxic effect, does not assemble into soluble oligomers. FEBS Lett (2008) 0.87
Evolution of genes involved in gamete interaction: evidence for positive selection, duplications and losses in vertebrates. PLoS One (2012) 0.87
3D mapping of glycogenosis-causing mutations in the large regulatory alpha subunit of phosphorylase kinase. Biochim Biophys Acta (2008) 0.87
A novel DNA repair enzyme containing RNA recognition, G-patch and specific splicing factor 45-like motifs in the protozoan parasite Toxoplasma gondii. Eur J Biochem (2002) 0.87
A concanavalin A-like lectin domain in the CHS1/LYST protein, shared by members of the BEACH family. Bioinformatics (2009) 0.86
The V(D)J recombination/DNA repair factor artemis belongs to the metallo-beta-lactamase family and constitutes a critical developmental checkpoint of the lymphoid system. Ann N Y Acad Sci (2003) 0.86
Non-intertwined binary patterns of hydrophobic/nonhydrophobic amino acids are considerably better markers of regular secondary structures than nonconstrained patterns. Proteins (2003) 0.85
ACCA phosphopeptide recognition by the BRCT repeats of BRCA1. J Mol Biol (2006) 0.85
Progress in gene delivery by cationic lipids: guanidinium-cholesterol-based systems as an example. Curr Drug Targets (2002) 0.85
Up date on IGFBP-4: regulation of IGFBP-4 levels and functions, in vitro and in vivo. Growth Horm IGF Res (2004) 0.85
Functional analysis of human RhCG: comparison with E. coli ammonium transporter reveals similarities in the pore and differences in the vestibule. Am J Physiol Cell Physiol (2009) 0.85
Synthesis and transfection activity of new cationic phosphoramidate lipids: high efficiency of an imidazolium derivative. Chembiochem (2008) 0.85
Gene transfer by chemical vectors, and endocytosis routes of polyplexes, lipoplexes and lipopolyplexes in a myoblast cell line. Biomaterials (2012) 0.85
Aminoglycoside-derived cationic lipids as efficient vectors for gene transfection in vitro and in vivo. J Gene Med (2002) 0.85
Kanamycin A-derived cationic lipids as vectors for gene transfection. Chembiochem (2005) 0.85
Characterization of novel RHD alleles: relationship between phenotype, genotype, and trimeric architecture. Transfusion (2012) 0.84
Toxoplasma gondii Alba proteins are involved in translational control of gene expression. J Mol Biol (2013) 0.83
Calcineurin B-like domains in the large regulatory alpha/beta subunits of phosphorylase kinase. Proteins (2008) 0.83
The ARRIVE guidelines, a welcome improvement to standards for reporting animal research. J Gene Med (2010) 0.83
[The active site of human glucocerebrosidase: structural predictions and experimental validations]. J Soc Biol (2002) 0.82
Comprehensive repertoire of foldable regions within whole genomes. PLoS Comput Biol (2013) 0.82
A novel cationic lipophosphoramide with diunsaturated lipid chains: synthesis, physicochemical properties, and transfection activities. J Med Chem (2010) 0.82
Bromodomain testis-specific protein is expressed in mouse oocyte and evolves faster than its ubiquitously expressed paralogs BRD2, -3, and -4. Genomics (2006) 0.82
1H, 13C and 15N resonance assignments of the region 1463-1617 of the mouse p53 binding protein 1 (53BP1). J Biomol NMR (2004) 0.82
The C-terminal extension of human RTEL1, mutated in Hoyeraal-Hreidarsson syndrome, contains harmonin-N-like domains. Proteins (2013) 0.82
Dombrock genotyping in a native Congolese cohort reveals two novel alleles. Transfusion (2009) 0.82
C terminus of nucleotide binding domain 1 contains critical features for cystic fibrosis transmembrane conductance regulator trafficking and activation. J Biol Chem (2010) 0.81
Striking conformational change suspected within the phosphoribulokinase dimer induced by interaction with GAPDH. J Biol Chem (2001) 0.81
The evolutionary history of the SAL1 gene family in eutherian mammals. BMC Evol Biol (2011) 0.81
Lipophosphonate/lipophosphoramidates: a family of synthetic vectors efficient for gene delivery. Biochimie (2011) 0.81
Viral fusion peptides and identification of membrane-interacting segments. Biochem Biophys Res Commun (2002) 0.80
Combined suicide gene therapy for pancreatic peritoneal carcinomatosis using BGTC liposomes. Cancer Gene Ther (2004) 0.80
Structure-function analysis of the human ferroportin iron exporter (SLC40A1): effect of hemochromatosis type 4 disease mutations and identification of critical residues. Hum Mutat (2013) 0.79
CFTR: effect of ICL2 and ICL4 amino acids in close spatial proximity on the current properties of the channel. J Cyst Fibros (2013) 0.79
Deficiency in Mucosa-associated Lymphoid Tissue Lymphoma Translocation 1: A Novel Cause of IPEX-Like Syndrome. J Pediatr Gastroenterol Nutr (2017) 0.79
Identification and characterization of Moca-cyp. A Drosophila melanogaster nuclear cyclophilin. J Biol Chem (2002) 0.79
The gene transfection properties of a lipophosphoramidate derivative with two phytanyl chains. Biomaterials (2012) 0.79
Identification of hidden relationships from the coupling of hydrophobic cluster analysis and domain architecture information. Bioinformatics (2013) 0.79
Munc13-4*rab27 complex tethers secretory lysosomes at the plasma membrane. Commun Integr Biol (2012) 0.78
Lipophosphoramidates as lipidic part of lipospermines for gene delivery. Bioconjug Chem (2007) 0.78
Folate-equipped pegylated archaeal lipid derivatives: synthesis and transfection properties. Chemistry (2008) 0.78
Integrin receptor specificity for human red cell ICAM-4 ligand. Critical residues for alphaIIbeta3 binding. Eur J Biochem (2004) 0.78
Folate-equipped nanolipoplexes mediated efficient gene transfer into human epithelial cells. Int J Mol Sci (2013) 0.78
Paromomycin and neomycin B derived cationic lipids: synthesis and transfection studies. J Control Release (2011) 0.77