Published in Orphanet J Rare Dis on June 07, 2014
Minimum data set for cystic fibrosis registry: a case study in iran. Acta Inform Med (2015) 0.81
The EMBARC European Bronchiectasis Registry: protocol for an international observational study. ERJ Open Res (2016) 0.80
Assessing the feasibility of a web-based registry for multiple orphan lung diseases: the Australasian Registry Network for Orphan Lung Disease (ARNOLD) experience. Orphanet J Rare Dis (2016) 0.75
Global impact of bronchiectasis and cystic fibrosis. Breathe (Sheff) (2016) 0.75
Nationwide genetic analysis for molecularly unresolved cystic fibrosis patients in a multiethnic society: implications for preconception carrier screening. Mol Genet Genomic Med (2017) 0.75
A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics (1959) 12.04
Evaluation of a new definition for chronic Pseudomonas aeruginosa infection in cystic fibrosis patients. J Cyst Fibros (2003) 2.82
European Epidemiologic Registry of Cystic Fibrosis (ERCF): comparison of major disease manifestations between patients with different classes of mutations. Pediatr Pulmonol (2001) 1.44
Clinical evaluation of the macroduct sweat collection system and conductivity analyzer in the diagnosis of cystic fibrosis. J Pediatr (1994) 1.33
Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS patient registry. Eur Respir J (2013) 1.27
Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet (2010) 1.22
The relative frequency of CFTR mutation classes in European patients with cystic fibrosis. J Cyst Fibros (2014) 1.00
The how (and why) of disease registers. Early Hum Dev (2010) 0.99
Using the national registry of HIV-infected veterans in research: lessons for the development of disease registries. J Clin Epidemiol (2001) 0.99
Using disease registries for pharmacoepidemiological research: a case study of data from a cystic fibrosis registry. Pharmacoepidemiol Drug Saf (2003) 0.86
Reference percentiles for FEV(1) and BMI in European children and adults with cystic fibrosis. Orphanet J Rare Dis (2012) 0.83
A short history of pathology registries, with emphasis on cancer registries. Soz Praventivmed (2003) 0.82
Longitudinal study of infectious intestinal disease in the UK (IID2 study): incidence in the community and presenting to general practice. Gut (2011) 3.71
Understanding the natural progression in %FEV1 decline in patients with cystic fibrosis: a longitudinal study. Thorax (2012) 3.08
European best practice guidelines for cystic fibrosis neonatal screening. J Cyst Fibros (2009) 1.75
Cystic fibrosis diagnosed after 2 months of age leads to worse outcomes and requires more therapy. Pediatrics (2007) 1.73
Economic implications of newborn screening for cystic fibrosis: a cost of illness retrospective cohort study. Lancet (2007) 1.66
Decreased lung function in female but not male subjects with established cystic fibrosis-related diabetes. Diabetes Care (2005) 1.56
Demographics of the UK cystic fibrosis population: implications for neonatal screening. Eur J Hum Genet (2002) 1.33
Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS patient registry. Eur Respir J (2013) 1.27
Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet (2010) 1.22
Mechanistic insight into control of CFTR by AMPK. J Biol Chem (2008) 1.21
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation. Autophagy (2014) 1.21
Liquid secretion inhibitors reduce mucociliary transport in glandular airways. Am J Physiol Lung Cell Mol Physiol (2002) 1.10
Expression of wild-type CFTR suppresses NF-kappaB-driven inflammatory signalling. PLoS One (2010) 1.08
Comparative analysis of Cystic Fibrosis Registry data from the UK with USA, France and Australasia. J Cyst Fibros (2005) 1.05
Clinical and sociodemographic study of vitiligo. Indian J Dermatol Venereol Leprol (2009) 0.99
Novel regulation of cystic fibrosis transmembrane conductance regulator (CFTR) channel gating by external chloride. J Biol Chem (2004) 0.99
Modulation of protein kinase CK2 activity by fragments of CFTR encompassing F508 may reflect functional links with cystic fibrosis pathogenesis. Biochemistry (2008) 0.98
Defective CFTR expression and function are detectable in blood monocytes: development of a new blood test for cystic fibrosis. PLoS One (2011) 0.98
Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries. J Cyst Fibros (2010) 0.98
CFTR: a hub for kinases and crosstalk of cAMP and Ca2+. FEBS J (2013) 0.95
Regulation of the epithelial Na+ channel by the protein kinase CK2. J Biol Chem (2008) 0.95
The formation of the cAMP/protein kinase A-dependent annexin 2-S100A10 complex with cystic fibrosis conductance regulator protein (CFTR) regulates CFTR channel function. Mol Biol Cell (2007) 0.95
Newborn screening for cystic fibrosis is associated with reduced treatment intensity. J Pediatr (2005) 0.95
Inhibition of protein kinase CK2 closes the CFTR Cl channel, but has no effect on the cystic fibrosis mutant deltaF508-CFTR. Cell Physiol Biochem (2009) 0.94
Making human nasal cilia beat in the cold: a real time assay for cell signalling. Cell Signal (2003) 0.92
The phosphorylation status of membrane-bound nucleoside diphosphate kinase in epithelia and the role of AMP. Mol Cell Biochem (2009) 0.91
Nucleoside diphosphate kinase A as a controller of AMP-kinase in airway epithelia. J Bioenerg Biomembr (2006) 0.90
Ancestral haplotype 8.1 and lung disease severity in European cystic fibrosis patients. J Cyst Fibros (2011) 0.88
Retracted Protein kinase CK2, cystic fibrosis transmembrane conductance regulator, and the deltaF508 mutation: F508 deletion disrupts a kinase-binding site. J Biol Chem (2007) 0.87
Retracted A novel physical and functional association between nucleoside diphosphate kinase A and AMP-activated protein kinase alpha1 in liver and lung. Biochem J (2005) 0.87
CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus vector. J Cell Physiol (2008) 0.87
Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity. Mol Cell Biol (2011) 0.87
Neonatal screening for cystic fibrosis is beneficial even in the context of modern treatment. J Pediatr (2005) 0.86
Evaluation of a home telemonitoring service for adult patients with cystic fibrosis: a pilot study. J Telemed Telecare (2010) 0.85
Detection of phospho-sites generated by protein kinase CK2 in CFTR: mechanistic aspects of Thr1471 phosphorylation. PLoS One (2013) 0.85
Asians with cystic fibrosis in the UK have worse disease outcomes than clinic matched white homozygous delta F508 controls. J Cyst Fibros (2005) 0.85
Delayed diagnosis of females with respiratory presentation of cystic fibrosis did not segregate with poorer clinical outcome. J Clin Epidemiol (2006) 0.85
Stomatin immunoreactivity in ciliated cells of the human airway epithelium. Anat Embryol (Berl) (2003) 0.84
Dictyostelium discoideum nucleoside diphosphate kinase C plays a negative regulatory role in phagocytosis, macropinocytosis and exocytosis. PLoS One (2011) 0.84
Cystic fibrosis transmembrane regulator fragments with the Phe508 deletion exert a dual allosteric control over the master kinase CK2. Biochem J (2010) 0.84
Retracted Understanding the molecular basis of the interaction between NDPK-A and AMPK alpha 1. Mol Cell Biol (2006) 0.84
NDPK-A (but not NDPK-B) and AMPK alpha1 (but not AMPK alpha2) bind the cystic fibrosis transmembrane conductance regulator in epithelial cell membranes. Cell Signal (2006) 0.83
Reference percentiles for FEV(1) and BMI in European children and adults with cystic fibrosis. Orphanet J Rare Dis (2012) 0.83
Overweight and obesity in deltaF508 homozygous cystic fibrosis. J Pediatr (2005) 0.82
Exercise and quality of life in patients with cystic fibrosis: A 12-week intervention study. Physiother Theory Pract (2011) 0.82
Small interfering peptide (siP) for in vivo examination of the developing lung interactonome. Dev Dyn (2009) 0.81
4alpha-Phorbol negates the inhibitory effects of phorbol-12-myristate-13-acetate on human cilia and alters the phosphorylation of PKC. FEBS Lett (2002) 0.81
Role of binding and nucleoside diphosphate kinase A in the regulation of the cystic fibrosis transmembrane conductance regulator by AMP-activated protein kinase. J Biol Chem (2012) 0.81
A methodology to establish a database to study gene environment interactions for childhood asthma. BMC Med Res Methodol (2010) 0.81
Gastrointestinal transit times and motility in patients with cystic fibrosis. Scand J Gastroenterol (2012) 0.81
Origins of chemical evolution. Acc Chem Res (2012) 0.81
CFTR mutations altering CFTR fragmentation. Biochem J (2013) 0.80
The NM23-H1/H2 homolog NDK-1 is required for full activation of Ras signaling in C. elegans. Development (2013) 0.80
Understanding protein kinase CK2 mis-regulation upon F508del CFTR expression. Naunyn Schmiedebergs Arch Pharmacol (2011) 0.79
Epithelial IgG and its relationship to the loss of F508 in the common mutant form of the cystic fibrosis transmembrane conductance regulator. FEBS Lett (2009) 0.79
A genetic interaction between NDPK and AMPK in Dictyostelium discoideum that affects motility, growth and development. Naunyn Schmiedebergs Arch Pharmacol (2011) 0.79
Transglutaminase 2 and nucleoside diphosphate kinase activity are correlated in epithelial membranes and are abnormal in cystic fibrosis. FEBS Lett (2009) 0.78
Metformin treatment of diabetes mellitus increases the risk for pancreatitis in patients bearing the CFTR-mutation S573C. Cell Physiol Biochem (2010) 0.77
Fertility and pregnancy outcomes in men and women with cystic fibrosis in the United Kingdom. Hum Reprod (2004) 0.77
Defective formation of PKA/CnA-dependent annexin 2-S100A10/CFTR complex in DeltaF508 cystic fibrosis cells. Cell Signal (2008) 0.77
Retracted Protein kinase CK2 acts as a signal molecule switching between the NDPK-A/AMPK alpha1 complex and NDPK-B. FASEB J (2006) 0.77
Protein kinase CK2 acts as a signal molecule switching between the NDPK-A/AMPK 1 complex and NDPK-B. FASEB J (2007) 0.76
Nucleoside diphosphate kinases (NDPKs) in animal development. Cell Mol Life Sci (2014) 0.76
The second study of infectious intestinal disease (IID2): increased rates of recurrent diarrhoea in individuals aged 65 years and above. BMC Public Health (2013) 0.75
Protein kinase CK2, cystic fibrosis transmembrane conductance regulator, and the deltaF508 mutation: F508 deletion disrupts a kinase-binding site. J Biol Chem (2008) 0.75