Published in Am J Respir Cell Mol Biol on April 03, 2009
Measurement of the airway surface liquid volume with simple light refraction microscopy. Am J Respir Cell Mol Biol (2011) 1.04
Control of TMEM16A by INO-4995 and other inositolphosphates. Br J Pharmacol (2013) 0.80
Mucociliary differentiation of serially passaged normal human tracheobronchial epithelial cells. Am J Respir Cell Mol Biol (1996) 4.34
New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J (2004) 3.87
Relative ion permeability of normal and cystic fibrosis nasal epithelium. J Clin Invest (1983) 2.72
Cystic fibrosis in adolescents and adults. Thorax (1987) 2.47
Structure, function and regulation of carboxylesterases. Chem Biol Interact (2006) 1.80
Regulation of airway surface liquid volume by human airway epithelia. Pflugers Arch (2002) 1.66
Regulation of airway surface liquid volume and mucus transport by active ion transport. Proc Am Thorac Soc (2004) 1.64
Regulation of murine airway surface liquid volume by CFTR and Ca2+-activated Cl- conductances. J Gen Physiol (2002) 1.41
Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis. Am J Respir Crit Care Med (2007) 1.32
The pathway for the production of inositol hexakisphosphate in human cells. J Biol Chem (2004) 1.26
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium. Proc Natl Acad Sci U S A (1997) 1.19
Multitasking in signal transduction by a promiscuous human Ins(3,4,5,6)P(4) 1-kinase/Ins(1,3,4)P(3) 5/6-kinase. Biochem J (2000) 1.16
Increased levels of inositol hexakisphosphate (InsP6) protect HEK293 cells from tumor necrosis factor (alpha)- and Fas-induced apoptosis. J Biol Chem (2005) 0.99
Properties of the inositol 3,4,5,6-tetrakisphosphate 1-kinase purified from rat liver. Regulation of enzyme activity by inositol 1,3,4-trisphosphate. J Biol Chem (1997) 0.93
Normalization of ion transport in murine cystic fibrosis nasal epithelium using gene transfer. Am J Physiol (1997) 0.92
Examining basal chloride transport using the nasal potential difference response in a murine model. Am J Physiol Lung Cell Mol Physiol (2001) 0.88
Relationships between cystic fibrosis transmembrane conductance regulator, extracellular nucleotides and cystic fibrosis. Pharmacol Ther (2006) 0.87
Phosphatidic acid modulates Cl- secretion in T84 cells: varying effects depending on mode of stimulation. Am J Physiol (1993) 0.83
Inositol polyphosphate derivative inhibits Na+ transport and improves fluid dynamics in cystic fibrosis airway epithelia. Am J Physiol Cell Physiol (2005) 0.81
Can intervention in inositol phosphate signalling pathways improve therapy for cystic fibrosis? Expert Opin Ther Targets (2005) 0.80
Well-differentiated human airway epithelial cell cultures. Methods Mol Med (2005) 4.66
Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2. Nat Genet (2011) 3.02
Mutations in INPP5E, encoding inositol polyphosphate-5-phosphatase E, link phosphatidyl inositol signaling to the ciliopathies. Nat Genet (2009) 2.72
Inositol polyphosphate 4-phosphatase II regulates PI3K/Akt signaling and is lost in human basal-like breast cancers. Proc Natl Acad Sci U S A (2010) 2.67
Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease. Nature (2009) 2.05
Genetic modifiers of liver disease in cystic fibrosis. JAMA (2009) 1.88
Phosphoinositide-specific inositol polyphosphate 5-phosphatase IV inhibits Akt/protein kinase B phosphorylation and leads to apoptotic cell death. J Biol Chem (2001) 1.64
Classifying severity of cystic fibrosis lung disease using longitudinal pulmonary function data. Am J Respir Crit Care Med (2006) 1.63
Coordinated release of nucleotides and mucin from human airway epithelial Calu-3 cells. J Physiol (2007) 1.59
Disruption of the mouse inositol 1,3,4,5,6-pentakisphosphate 2-kinase gene, associated lethality, and tissue distribution of 2-kinase expression. Proc Natl Acad Sci U S A (2005) 1.37
Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice. Am J Physiol Lung Cell Mol Physiol (2004) 1.33
The inositol polyphosphate 5-phosphatase Ocrl associates with endosomes that are partially coated with clathrin. Proc Natl Acad Sci U S A (2004) 1.32
Bestrophin-2 mediates bicarbonate transport by goblet cells in mouse colon. J Clin Invest (2010) 1.29
The synthesis of inositol hexakisphosphate. Characterization of human inositol 1,3,4,5,6-pentakisphosphate 2-kinase. J Biol Chem (2002) 1.27
The pathway for the production of inositol hexakisphosphate in human cells. J Biol Chem (2004) 1.26
Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis. Nat Genet (2012) 1.26
The mRNA export factor human Gle1 interacts with the nuclear pore complex protein Nup155. Mol Cell Proteomics (2003) 1.25
The human homolog of the rat inositol phosphate multikinase is an inositol 1,3,4,6-tetrakisphosphate 5-kinase. J Biol Chem (2002) 1.21
Characterization of myotubularin-related protein 7 and its binding partner, myotubularin-related protein 9. Proc Natl Acad Sci U S A (2003) 1.20
Identification of myotubularin as the lipid phosphatase catalytic subunit associated with the 3-phosphatase adapter protein, 3-PAP. Proc Natl Acad Sci U S A (2003) 1.19
Inositol 1,3,4-trisphosphate 5/6-kinase associates with the COP9 signalosome by binding to CSN1. J Biol Chem (2002) 1.18
Specificity determinants in inositol polyphosphate synthesis: crystal structure of inositol 1,3,4-trisphosphate 5/6-kinase. Mol Cell (2005) 1.18
The identification and characterization of two phosphatidylinositol-4,5-bisphosphate 4-phosphatases. Proc Natl Acad Sci U S A (2005) 1.11
Type I phosphatidylinositol-4,5-bisphosphate 4-phosphatase regulates stress-induced apoptosis. Proc Natl Acad Sci U S A (2007) 1.10
SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis. Am J Respir Cell Mol Biol (2005) 1.08
Regulation of PI(3)K/Akt signalling and cellular transformation by inositol polyphosphate 4-phosphatase-1. EMBO Rep (2009) 1.04
Generation of a conditional null allele for Cftr in mice. Genesis (2008) 1.00
A novel lung disease phenotype adjusted for mortality attrition for cystic fibrosis genetic modifier studies. Pediatr Pulmonol (2011) 1.00
Increased levels of inositol hexakisphosphate (InsP6) protect HEK293 cells from tumor necrosis factor (alpha)- and Fas-induced apoptosis. J Biol Chem (2005) 0.99
Mouse as a model of growth retardation in cystic fibrosis. Pediatr Res (2006) 0.98
Oxidative stress causes IL8 promoter hyperacetylation in cystic fibrosis airway cell models. Am J Respir Cell Mol Biol (2008) 0.97
Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium. Am J Physiol Gastrointest Liver Physiol (2011) 0.97
Cse1l is a negative regulator of CFTR-dependent fluid secretion. Curr Biol (2010) 0.96
Genetic modifiers of cystic fibrosis-related diabetes. Diabetes (2013) 0.96
MTMR9 increases MTMR6 enzyme activity, stability, and role in apoptosis. J Biol Chem (2008) 0.94
Preclinical MR fingerprinting (MRF) at 7 T: effective quantitative imaging for rodent disease models. NMR Biomed (2015) 0.93
Mucin variable number tandem repeat polymorphisms and severity of cystic fibrosis lung disease: significant association with MUC5AC. PLoS One (2011) 0.92
Infertility in females with cystic fibrosis is multifactorial: evidence from mouse models. Endocrinology (2008) 0.92
Myotubularin-related protein (MTMR) 9 determines the enzymatic activity, substrate specificity, and role in autophagy of MTMR8. Proc Natl Acad Sci U S A (2012) 0.91
Neural tube defects in mice with reduced levels of inositol 1,3,4-trisphosphate 5/6-kinase. Proc Natl Acad Sci U S A (2009) 0.90
Histo-blood group gene polymorphisms as potential genetic modifiers of infection and cystic fibrosis lung disease severity. PLoS One (2009) 0.89
Inositol 1,3,4-trisphosphate 5/6-kinase inhibits tumor necrosis factor-induced apoptosis. J Biol Chem (2003) 0.89
Inositol polyphosphate 4-phosphatase B as a regulator of bone mass in mice and humans. Cell Metab (2011) 0.89
Genetic influences on cystic fibrosis lung disease severity. Front Pharmacol (2013) 0.88
Age of Pseudomonas aeruginosa acquisition and subsequent severity of cystic fibrosis lung disease. Pediatr Pulmonol (2010) 0.87
EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity. Physiol Genomics (2009) 0.87
Variation in MSRA modifies risk of neonatal intestinal obstruction in cystic fibrosis. PLoS Genet (2012) 0.86
Beta 2 adrenergic receptor polymorphisms in cystic fibrosis. Pediatr Pulmonol (2005) 0.85
Some like it hot: curcumin and CFTR. Trends Mol Med (2004) 0.84
ADEMA: an algorithm to determine expected metabolite level alterations using mutual information. PLoS Comput Biol (2013) 0.83
Inositol polyphosphate multikinase regulates inositol 1,4,5,6-tetrakisphosphate. Biochem Biophys Res Commun (2005) 0.83
Phospholipase C-β3 is a key modulator of IL-8 expression in cystic fibrosis bronchial epithelial cells. J Immunol (2011) 0.82
Loss of CFTR results in reduction of histone deacetylase 2 in airway epithelial cells. Am J Physiol Lung Cell Mol Physiol (2009) 0.81
Inositol polyphosphate derivative inhibits Na+ transport and improves fluid dynamics in cystic fibrosis airway epithelia. Am J Physiol Cell Physiol (2005) 0.81
The role of myotubularin-related phosphatases in the control of autophagy and programmed cell death. Adv Biol Regul (2012) 0.80
Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice. J Cyst Fibros (2013) 0.80
Strain-specific variants of the mouse Cftr promoter region reveal transcriptional regulatory elements. Hum Mol Genet (2004) 0.79
Altered de novo lipogenesis contributes to low adipose stores in cystic fibrosis mice. Am J Physiol Gastrointest Liver Physiol (2012) 0.79
Blasts from the past. J Clin Invest (2004) 0.78
Regulation of inositol 1,3,4-trisphosphate 5/6-kinase (ITPK1) by reversible lysine acetylation. Proc Natl Acad Sci U S A (2012) 0.77
Collection of islets of Langerhans using an equilibrium method. Biotechniques (2013) 0.76
Epithelial monolayer culture system for real-time single-cell analyses. Physiol Rep (2014) 0.76
Characterization of tissue-specific and developmentally regulated alternative splicing of exon 64 in the COL5A1 gene. Connect Tissue Res (2011) 0.75
The role of inositol polyphosphate 4-phosphatase 1 in platelet function using a weeble mouse model. Adv Enzyme Regul (2010) 0.75
HIPAA strengthens business case for electronic report distribution systems. J Healthc Inf Manag (2002) 0.75
Corrigendum: Novel variation at chr11p13 associated with cystic fibrosis lung disease severity. Hum Genome Var (2017) 0.75