Cystic fibrosis (CF) is a common autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) (1). CF affects multiple organs, including lungs, pancreas, intestine, liver, sweat glands, gall bladder and male genital tract. Airway infection and inflammation cun'ently cause most of the morbidity and mortality. Although several therapies have improved the lives of patients, current treatments are inadequate and CF remains a lethal disease. Our knowledge about the pathogenesis of the disease, its progression, and the state of the neonatal lung is inadequate. Moreover, despite the potential of gene transfer to be an effective treatment, some of the most fundamental questions remain unanswered. These gaps in our knowledge have hindered attempts to develop better treatments and preventions for CF lung disease. A major impediment to addressing these issues has been limitations of current animal models. Although mouse strains carrying null and missense CFTR mutations have made enormous contributions, CF mice do not develop the airway or pancreatic disease typically found in humans.
| Lynch, Thomas J; Anderson, Preston J; Rotti, Pavana G et al. (2018) Submucosal Gland Myoepithelial Cells Are Reserve Stem Cells That Can Regenerate Mouse Tracheal Epithelium. Cell Stem Cell 22:653-667.e5 |
| Meyerholz, David K; Stoltz, David A; Gansemer, Nick D et al. (2018) Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs. Lab Invest 98:825-838 |
| Gray, Robert D; Hardisty, Gareth; Regan, Kate H et al. (2018) Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis. Thorax 73:134-144 |
| Thornell, Ian M; Li, Xiaopeng; Tang, Xiao Xiao et al. (2018) Nominal carbonic anhydrase activity minimizes airway-surface liquid pH changes during breathing. Physiol Rep 6: |
| Reznikov, Leah R; Meyerholz, David K; Abou Alaiwa, Mahmoud et al. (2018) The vagal ganglia transcriptome identifies candidate therapeutics for airway hyperreactivity. Am J Physiol Lung Cell Mol Physiol 315:L133-L148 |
| Meyerholz, David K; Beck, Amanda P; Goeken, J Adam et al. (2018) Glycogen depletion can increase the specificity of mucin detection in airway tissues. BMC Res Notes 11:763 |
| O'Malley, Yunxia; Rotti, Pavana G; Thornell, Ian M et al. (2018) Development of a polarized pancreatic ductular cell epithelium for physiological studies. J Appl Physiol (1985) 125:97-106 |
| Rosen, Bradley H; Chanson, Marc; Gawenis, Lara R et al. (2018) Animal and model systems for studying cystic fibrosis. J Cyst Fibros 17:S28-S34 |
| Meyerholz, David K; Sieren, Jessica C; Beck, Amanda P et al. (2018) Approaches to Evaluate Lung Inflammation in Translational Research. Vet Pathol 55:42-52 |
| Rosen, Bradley H; Evans, T Idil Apak; Moll, Shashanna R et al. (2018) Infection Is Not Required for Mucoinflammatory Lung Disease in CFTR-Knockout Ferrets. Am J Respir Crit Care Med 197:1308-1318 |
Showing the most recent 10 out of 184 publications
