Mouse models have become increasingly valuable for biomedical research, and their importance is particularly relevant to cystic fibrosis science as attested to by scores of publications describing studies performed on these models as a means to understand and treat the human disease. The Animal Models Core Facility provides a variety of mouse lines to CF P30 investigators, including previously generated and characterized strains that include the UNC (Cftr[tm1Unc]) and Cambridge (Cftr[tm1Cam]) Cftr knockout;Cftr-G551D (Cftr[Tm1G551D]);Cftr-F508del line (Cftr[Tm1Kth]);a transgenic (Tg) line expressing wild type human CFTR on a Cftr[-/-] background (hCFTR Cftr[-/-]), and a similar Tg mouse line expressing a human CFTR-G542X on a Cftr[-/-] background (hCFTR-G542X Cftr[-/-]). In addition to these established mouse lines available at our Center, we have assisted P30 investigators with the generation and/or breeding of several novel CF mouse models, including a Cftr-G542X knock-in line (Cftr[Tm1G542X]) and a Tg line expressing human CFTR-W1282X in a Cftr[-/-] background (hCFTR-W1282X Cftr[-/-]). A number of other murine lines are presently under construction in Core B. The Animal Models Core also assists CF investigators by providing functional CFTR assays, including nasal Potential Difference (PD) measurements assays in live animals as well as intestinal short circuit current assays of freshly excised tissues. The overall purpose of the Animal Models Core is to provide all necessary murine resources for the successful completion of individual P30 projects, and to perform advanced physiologic and other testing of CF animal models. These resources allow investigators to examine CF disease mechanism in vivo, and facilitate preclinical evaluation of experimental therapeutics for the disease. Thus, the Animal Models Core is an indispensable component of the overall P30.
Core B provides animal models, electrophysiologic equipment, solutions, and resources to be shared among many investigators without the need to duplicate the same capabilities in multiple laboratories. The Core has been indispensable for research priorities delineated by the overall UAB P30, including studies of CFTR cellular biology, tissue pathogenesis, and clinical translation.
| Shei, Ren-Jay; Peabody, Jacelyn E; Kaza, Niroop et al. (2018) The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis. Curr Opin Pharmacol 43:152-165 |
| Gebert, Magdalena; Bartoszewska, Sylwia; Janaszak-Jasiecka, Anna et al. (2018) PIWI proteins contribute to apoptosis during the UPR in human airway epithelial cells. Sci Rep 8:16431 |
| Montoro, Daniel T; Haber, Adam L; Biton, Moshe et al. (2018) A revised airway epithelial hierarchy includes CFTR-expressing ionocytes. Nature 560:319-324 |
| Lutful Kabir, Farruk; Ambalavanan, Namasivayam; Liu, Gang et al. (2018) MicroRNA-145 Antagonism Reverses TGF-? Inhibition of F508del CFTR Correction in Airway Epithelia. Am J Respir Crit Care Med 197:632-643 |
| Shei, Ren-Jay; Peabody, Jacelyn E; Rowe, Steven M (2018) Functional Anatomic Imaging of the Airway Surface. Ann Am Thorac Soc 15:S177-S183 |
| Clancy, John Paul; Cotton, Calvin U; Donaldson, Scott H et al. (2018) CFTR modulator theratyping: Current status, gaps and future directions. J Cyst Fibros : |
| Plyler, Z E; Birket, S E; Schultz, B D et al. (2018) Non-obstructive vas deferens and epididymis loss in cystic fibrosis rats. Mech Dev : |
| Poore, T Spencer; Virella-Lowell, Isabel; Guimbellot, Jennifer S (2018) Potential pathogenicity of Inquilinus limosus in a pediatric patient with cystic fibrosis. Pediatr Pulmonol 53:E21-E23 |
| Heltshe, Sonya L; Rowe, Steven M; Skalland, Michelle et al. (2018) Ivacaftor-treated Patients with Cystic Fibrosis Derive Long-Term Benefit Despite No Short-Term Clinical Improvement. Am J Respir Crit Care Med 197:1483-1486 |
| Guimbellot, Jennifer; Solomon, George M; Baines, Arthur et al. (2018) Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations. J Cyst Fibros : |
Showing the most recent 10 out of 175 publications
