Published in J Biol Chem on March 04, 2008
CFTR is a negative regulator of NFkappaB mediated innate immune response. PLoS One (2009) 1.57
Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD. Chest (2013) 1.32
Mechanisms of the noxious inflammatory cycle in cystic fibrosis. Respir Res (2009) 1.16
Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells. Am J Respir Cell Mol Biol (2009) 1.12
β-cell dysfunctional ERAD/ubiquitin/proteasome system in type 2 diabetes mediated by islet amyloid polypeptide-induced UCH-L1 deficiency. Diabetes (2010) 1.10
Potential for therapeutic manipulation of the UPR in disease. Semin Immunopathol (2013) 0.99
Cigarette smoke and CFTR: implications in the pathogenesis of COPD. Am J Physiol Lung Cell Mol Physiol (2013) 0.96
Glucosamine-induced endoplasmic reticulum stress affects GLUT4 expression via activating transcription factor 6 in rat and human skeletal muscle cells. Diabetologia (2010) 0.95
The silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunction. FASEB J (2013) 0.93
Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator. Expert Rev Proteomics (2010) 0.89
The calpain, caspase 12, caspase 3 cascade leading to apoptosis is altered in F508del-CFTR expressing cells. PLoS One (2009) 0.88
The UPR and lung disease. Semin Immunopathol (2013) 0.86
PGC-1α Promoter Methylation in Parkinson's Disease. PLoS One (2015) 0.85
Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1. Br J Pharmacol (2011) 0.85
Multiple mechanisms influence regulation of the cystic fibrosis transmembrane conductance regulator gene promoter. Am J Respir Cell Mol Biol (2009) 0.83
UCHL1 deficiency exacerbates human islet amyloid polypeptide toxicity in β-cells: evidence of interplay between the ubiquitin/proteasome system and autophagy. Autophagy (2014) 0.83
The unfolded protein response affects readthrough of premature termination codons. EMBO Mol Med (2014) 0.82
The yin and yang of cystic fibrosis transmembrane conductance regulator function: implications for chronic lung disease. Am J Respir Crit Care Med (2013) 0.81
Genetic susceptibility for chronic bronchitis in chronic obstructive pulmonary disease. Respir Res (2014) 0.80
Regulation of the unfolded protein response by microRNAs. Cell Mol Biol Lett (2013) 0.79
Assay strategies for identification of therapeutic leads that target protein trafficking. Trends Pharmacol Sci (2015) 0.78
Microparticle-mediated transfer of the viral receptors CAR and CD46, and the CFTR channel in a CHO cell model confers new functions to target cells. PLoS One (2012) 0.77
VAMP-associated Proteins (VAP) as Receptors That Couple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Proteostasis with Lipid Homeostasis. J Biol Chem (2016) 0.75
Phenotypically concordant and discordant monozygotic twins display different DNA copy-number-variation profiles. Am J Hum Genet (2008) 5.53
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations. N Engl J Med (2003) 4.43
LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies. Nat Med (2004) 3.00
Position paper for the organization of extracorporeal membrane oxygenation programs for acute respiratory failure in adult patients. Am J Respir Crit Care Med (2014) 2.78
Molecular recognition by LARGE is essential for expression of functional dystroglycan. Cell (2004) 2.33
Somatic mosaicism for copy number variation in differentiated human tissues. Hum Mutat (2008) 2.30
Age-related somatic structural changes in the nuclear genome of human blood cells. Am J Hum Genet (2012) 2.15
Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression. Am J Physiol Cell Physiol (2006) 1.82
Exon array CGH: detection of copy-number changes at the resolution of individual exons in the human genome. Am J Hum Genet (2005) 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
A full-coverage, high-resolution human chromosome 22 genomic microarray for clinical and research applications. Hum Mol Genet (2002) 1.69
The signal transducers Stat1 and Stat3 and their novel target Jmjd3 drive the expression of inflammatory genes in microglia. J Mol Med (Berl) (2013) 1.64
Genomic microarrays in the spotlight. Trends Genet (2004) 1.55
Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones. Biochem J (2008) 1.49
Profiling of copy number variations (CNVs) in healthy individuals from three ethnic groups using a human genome 32 K BAC-clone-based array. Hum Mutat (2008) 1.45
Activation of the unfolded protein response by deltaF508 CFTR. Am J Respir Cell Mol Biol (2008) 1.41
High-resolution DNA copy number profiling of malignant peripheral nerve sheath tumors using targeted microarray-based comparative genomic hybridization. Clin Cancer Res (2008) 1.34
Venoarterial extracorporeal membrane oxygenation (VA-ECMO) in pediatric cardiac support. Ann Thorac Surg (2006) 1.17
Regulation of amiloride-sensitive Na(+) transport by basal nitric oxide. Am J Respir Cell Mol Biol (2003) 1.13
Continuous renal replacement therapy for children ≤10 kg: a report from the prospective pediatric continuous renal replacement therapy registry. J Pediatr (2012) 1.12
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
Hesperidin stimulates cystic fibrosis transmembrane conductance regulator-mediated chloride secretion and ciliary beat frequency in sinonasal epithelium. Otolaryngol Head Neck Surg (2010) 1.11
Restoration of W1282X CFTR activity by enhanced expression. Am J Respir Cell Mol Biol (2007) 1.10
Chromosome 22 array-CGH profiling of breast cancer delimited minimal common regions of genomic imbalances and revealed frequent intra-tumoral genetic heterogeneity. Int J Oncol (2006) 1.09
A segmental maximum a posteriori approach to genome-wide copy number profiling. Bioinformatics (2008) 1.09
Resveratrol has salutary effects on mucociliary transport and inflammation in sinonasal epithelium. Laryngoscope (2011) 1.09
2012 white paper on recent issues in bioanalysis and alignment of multiple guidelines. Bioanalysis (2012) 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
DNA copy-number analysis of the 22q11 deletion-syndrome region using array-CGH with genomic and PCR-based targets. Int J Mol Med (2004) 1.07
Microarray-based survey of CpG islands identifies concurrent hyper- and hypomethylation patterns in tissues derived from patients with breast cancer. Genes Chromosomes Cancer (2006) 1.05
Development of NF2 gene specific, strictly sequence defined diagnostic microarray for deletion detection. J Mol Med (Berl) (2003) 1.05
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
Common pathogenetic mechanism involving human chromosome 18 in familial and sporadic ileal carcinoid tumors. Genes Chromosomes Cancer (2011) 1.03
A previously unrecognized microdeletion syndrome on chromosome 22 band q11.2 encompassing the BCR gene. Am J Med Genet A (2007) 1.02
Characterization of novel and complex genomic aberrations in glioblastoma using a 32K BAC array. Neuro Oncol (2009) 1.02
Structural genetic variation in the context of somatic mosaicism. Methods Mol Biol (2012) 1.02
The bioflavonoid compound, sinupret, stimulates transepithelial chloride transport in vitro and in vivo. Laryngoscope (2010) 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
Workshop report: Crystal City V--quantitative bioanalytical method validation and implementation: the 2013 revised FDA guidance. AAPS J (2014) 0.99
Focal amplifications are associated with high grade and recurrences in stage Ta bladder carcinoma. Int J Cancer (2010) 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
Analysis of copy number variation in the normal human population within a region containing complex segmental duplications on 22q11 using high-resolution array-CGH. Genomics (2006) 0.98
Cigarette smoke and CFTR: implications in the pathogenesis of COPD. Am J Physiol Lung Cell Mol Physiol (2013) 0.96
The transcriptional map of the common eliminated region 1 (C3CER1) in 3p21.3. Eur J Hum Genet (2002) 0.96
Somatic mosaicism for chromosome X and Y aneuploidies in monozygotic twins heterozygous for sickle cell disease mutation. Am J Med Genet A (2010) 0.95
Regulation of angiogenesis by hypoxia: the role of microRNA. Cell Mol Biol Lett (2012) 0.94
Integrative epigenomic and genomic analysis of malignant pheochromocytoma. Exp Mol Med (2010) 0.93
Comprehensive genetic and epigenetic analysis of sporadic meningioma for macro-mutations on 22q and micro-mutations within the NF2 locus. BMC Genomics (2007) 0.93
Work and social adjustment in patients with anorexia nervosa. Compr Psychiatry (2012) 0.92
Identification of genetic aberrations on chromosome 22 outside the NF2 locus in schwannomatosis and neurofibromatosis type 2. Hum Mutat (2005) 0.92
Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator. Biochem J (2012) 0.92
Frequent genetic differences between matched primary and metastatic breast cancer provide an approach to identification of biomarkers for disease progression. Eur J Hum Genet (2010) 0.92
High-resolution gene copy number and expression profiling of human chromosome 22 in ovarian carcinomas. Genes Chromosomes Cancer (2005) 0.91
Comprehensive DNA copy number profiling of meningioma using a chromosome 1 tiling path microarray identifies novel candidate tumor suppressor loci. Cancer Res (2005) 0.91
CFTR expression regulation by the unfolded protein response. Methods Enzymol (2011) 0.91
Recurrent genomic alterations in benign and malignant pheochromocytomas and paragangliomas revealed by whole-genome array comparative genomic hybridization analysis. Endocr Relat Cancer (2010) 0.89
Chromosome 22 tiling-path array-CGH analysis identifies germ-line- and tumor-specific aberrations in patients with glioblastoma multiforme. Genes Chromosomes Cancer (2005) 0.89
Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator. Expert Rev Proteomics (2010) 0.89
Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array. Genes Chromosomes Cancer (2009) 0.88
High-resolution array-CGH profiling of germline and tumor-specific copy number alterations on chromosome 22 in patients affected with schwannomas. Hum Genet (2005) 0.88
Novel regulation of cell [Na(+)] in macula densa cells: apical Na(+) recycling by H-K-ATPase. Am J Physiol Renal Physiol (2002) 0.88
Hexose phosphorylation and the putative calcium channel component Mid1p are required for the hexose-induced transient elevation of cytosolic calcium response in Saccharomyces cerevisiae. Mol Microbiol (2002) 0.88
Comparative human/murine sequence analysis of the common eliminated region 1 from human 3p21.3. Mamm Genome (2002) 0.87
Distal 22q11.2 microduplication encompassing the BCR gene. Am J Med Genet A (2008) 0.86
Transient transfection of polarized epithelial monolayers with CFTR and reporter genes using efficacious lipids. Am J Physiol Cell Physiol (2002) 0.85
Search and rescue: finding ways to correct deltaF508 CFTR. Am J Respir Cell Mol Biol (2009) 0.85
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies. Cell Physiol Biochem (2005) 0.84
VCP/p97 AAA-ATPase does not interact with the endogenous wild-type cystic fibrosis transmembrane conductance regulator. Am J Respir Cell Mol Biol (2007) 0.83
Copy-number polymorphisms: mining the tip of an iceberg. Trends Genet (2005) 0.83
Coincidence of synteny breakpoints with malignancy-related deletions on human chromosome 3. Proc Natl Acad Sci U S A (2003) 0.83
Does chromosome 22 have anything to do with sex determination: further studies on a 46,XX,22q11.2 del male. Am J Med Genet A (2003) 0.81
O-GlcNAc modification of proteins affects volume regulation in Jurkat cells. Eur Biophys J (2009) 0.81
Regeneration of comparative genomic hybridization oligonucleotide microarrays with dimethylurea. Anal Biochem (2012) 0.80
Palindrome-mediated and replication-dependent pathogenic structural rearrangements within the NF1 gene. Hum Mutat (2014) 0.80
A patient with 22q11.2 deletion and Opitz syndrome-like phenotype has the same deletion as velocardiofacial patients. Am J Med Genet A (2007) 0.80
High-resolution profiling of an 11 Mb segment of human chromosome 22 in sporadic schwannoma using array-CGH. Int J Oncol (2003) 0.80
Risk factors leading to failed procedural sedation in children outside the operating room. Pediatr Emerg Care (2014) 0.79
Informed consent to participate in a research study -- gene therapy for cystic fibrosis using cationic liposome mediated gene transfer: a phase I trial of safety and efficacy in the nasal airway. Hum Gene Ther (1994) 0.78