CF And Effects of Drugs Mixed Ex Vivo With Sputum for Mucolytic Treatment to Lung Mucin
The investigators will collect samples of sputum from healthy volunteers and patients with cystic fibrosis for the purpose of: a) purifying airway mucins for plate-based binding studies and; b) assessment of the effects of carbohydrates on the rheologic properties of the sputum. This study has two hypotheses: 1. Lectins from Pseudomonas aeruginosa and Aspergillus fumigatus bind to airway mucins in a fucose-dependent manner, and this binding can be inhibited by fucosyl glycomimetic compounds. 2. Fucosyl glycomimetics will compete with Pseudomonas aeruginosa lectin (PA-IIL) and Aspergillus fumigatus lectin (AFL) and disrupt lectin-driven mucin cross-linking in CF sputum.
• Healthy control subjects:
‣ Age 18-65
⁃ No history of lung disease
• Cystic fibrosis subjects:
‣ Age 18-65
⁃ No history of lung disease other than cystic fibrosis
⁃ Diagnosis of CF if sweat chloride values \> 60 mM on two separate pilocarpine iontophoresis sweat tests and/or two allelic CF-producing mutations in genetic analysis