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Extracellular UDP-sugars promote cellular responses by interacting with widely distributed P2Y(14) receptors, but the mechanisms by which these molecules are released from cells are poorly understood. Given the active role of UDP-sugars in glycosylation reactions within the secretory pathway, we hypothesized that UDP-sugar release includes an exocytotic(More)
CFTR in cystic fibrosis F508 ∆ Potentiator ivacaftor abrogates pharmacological correction of Editor's Summary potentiators make CFTR less stable, accelerating the removal of this channel from the cell membrane. and Veit and coauthors now show that combining the two types of drugs does not work effectively, because surface. Other drugs, called(More)
BACKGROUND Although nontuberculous mycobacteria (NTM) are recognized pathogens in cystic fibrosis (CF), associations with clinical outcomes remain unclear. METHODS Microbiological data was obtained from 1216 CF patients over 8years (481+/-55patients/year). Relationships to clinical outcomes were examined in the subset (n=271, 203+/-23 patients/year) with(More)
Although nontuberculous mycobacteria (NTM) are recognized pathogens in adolescent and adult patients with cystic fibrosis (CF), the role of NTM in younger CF patients is not well-defined. To explore NTM infection in CF patients less than 12 years old, a retrospective review was performed. Prevalence was estimated from routine mycobacterial cultures of(More)
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR). Newly developed "correctors" such as lumacaftor (VX-809) that improve CFTR maturation and trafficking and "potentiators" such as ivacaftor (VX-770) that enhance channel activity may provide important advances in CF therapy. Although VX-770 has demonstrated(More)
Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilation of the normal lung lymphatics. The disease was previously associated with a poor prognosis. However, several recent case reports and case studies indicate that survival is possible in the severe neonatal form of PL, and the prognosis is good for infants who present after the(More)
Extracellular UDP-glucose is a natural purinergic receptor agonist, but its mechanisms of cellular release remain unclear. We studied these mechanisms in Saccharomyces cerevisiae, a simple model organism that releases ATP, another purinergic agonist. Similar to ATP, UDP-glucose was released by S. cerevisiae at a rate that was linear over time. However,(More)
Rapidly growing mycobacteria (RGM) are respiratory pathogens in patients with cystic fibrosis (CF), but detection generally requires specialized cultures for acid-fast bacilli (AFB; AFB cultures). We determined that RGM could be recovered from routine cultures of samples from patients with CF by extending incubation of the Burkholderia cepacia selective(More)
Exhaled breath condensate (EBC) analyses promise simple and noninvasive methods to measure airway biomarkers but pose considerable methodological challenges. We utilized mass spectrometry to measure EBC purine biomarkers adenosine and AMP plus urea to control for dilutional variability in two studies: 1) a cross-sectional analysis of 28 healthy, 40 cystic(More)
BACKGROUND Adenosine and related purines have established roles in inflammation, and elevated airway concentrations are predicted in patients with COPD. However, accurate airway surface purine measurements can be confounded by stimulation of purine release during collection of typical respiratory samples. METHODS Airway samples were collected(More)