caa a22 4500 475774027 CHVBK 20180406123625.0 cr unu---uuuuu 170329e20000701xx s 000 0 eng 10.1007/s004180000164 doi (NATIONALLICENCE)springer-10.1007/s004180000164 Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract [Elektronische Daten] [Nadia Ameen, John Alexis, Pedro Salas] Abstract.: The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP and cGMP-regulated chloride channel critical to the regulation of intestinal fluid, chloride, and bicarbonate secretion. In cystic fibrosis (CF), mutations in CFTR result in downregulation of CFTR function and small intestinal obstruction. Unlike the human CF intestine, severe gastrointestinal disease and lethal obstruction is common in transgenic mice deficient in CFTR. The relevance of the physiology of CFTR and pathophysiology of CF in genetically altered mice to that of human CF disease remains incompletely understood. We hypothesized that the expression and distribution of CFTR in mouse intestine may differ from that of human and may contribute to the variation in disease expression between the two species. Using immunocytochemical and immunoblot techniques and well-characterized anti-rodent anti-CFTR antibodies, we examined the cellular distribution of CFTR in the mouse intestinal tract. We identified significant differences in villus distribution for CFTR in the mouse proximal small intestine compared to those previously reported for human and rat. These observations are important to the understanding of CFTR pathophysiology in transgenic CF mouse model systems and bear relevance to the different phenotypic expression of disease in mice compared to human. Springer-Verlag, 2000 Mouse Intestine CFTR Localization Protein nationallicence Ameen Nadia Department of Pediatrics, University of Miami School of Medicine, MCCD, 6006B, 1601, NW 12th Avenue, Miami, FL 33136, USA aut Alexis John Department of Pathology, Mt. Sinai Medical Center, Miami Beach, Florida aut Salas Pedro Department of Cell Biology, University of Miami School of Medicine, Miami Beach, Florida, USA aut https://doi.org/10.1007/s004180000164 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s004180000164 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Ameen Nadia Department of Pediatrics, University of Miami School of Medicine, MCCD, 6006B, 1601, NW 12th Avenue, Miami, FL 33136, USA aut NATIONALLICENCE 700 1- Alexis John Department of Pathology, Mt. Sinai Medical Center, Miami Beach, Florida aut NATIONALLICENCE 700 1- Salas Pedro Department of Cell Biology, University of Miami School of Medicine, Miami Beach, Florida, USA aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer