Salivary secretion assay for drug efficacy for cystic fibrosis in mice

Intestinal bicarbonate secretion in cystic fibrosis mice.

Gene-targeted disruption of the cystic fibrosis transmembrane conductance regulator (CFTR) in mice results in an intestinal disease phenotype that is remarkably similar to bowel disease in cystic fibrosis patients. In the intestinal segment downstream from the stomach (i.e., the duodenum), CFTR plays an important role in bicarbonate secretion that protects the epithelium from acidic gastric eff...

Resveratrol increases F508del-CFTR dependent salivary secretion in cystic fibrosis mice

Cystic fibrosis (CF) is a fatal genetic disease associated with widespread exocrine gland dysfunction. Studies have suggested activating effects of resveratrol, a naturally-occurring polyphenol compound with antioxidant and anti-inflammatory properties, on CF transmembrane conductance regulator (CFTR) protein function. We assayed, in F508del-CFTR homozygous (CF) and in wild-type mice, the effec...

Biomarkers for cystic fibrosis drug development.

PURPOSE To provide a review of the status of biomarkers in cystic fibrosis drug development, including regulatory definitions and considerations, a summary of biomarkers in current use with supportive data, current gaps, and future needs. METHODS Biomarkers are considered across several areas of CF drug development, including cystic fibrosis transmembrane conductance regulator modulation, inf...

Hyperbranched polymer drug delivery treatment for lung metastasis of salivary adenoid cystic carcinoma in nude mice

Salivary adenoid cystic carcinoma (SACC) is associated with a high rate of lung metastasis. When lung metastasis occurs, the effects of traditional chemotherapy on SACC are poor. Hyperbranched polymer drug delivery (degradable hyperbranched polyglycerols, dHPGs) can be used as a strategy to load several drugs, and obtain beneficial effects on SACC lung metastasis through enhanced permeability a...

Bile acid secretion in cystic fibrosis.