Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator
JT Fisher, X Liu, Z Yan, M Luo, Y Zhang, W Zhou… - Journal of Biological …, 2012 - ASBMB
The most common cystic fibrosis transmembrane conductance regulator (CFTR) gene
mutation is ΔF508, and this causes cystic fibrosis (CF). New CF models in the pig and ferret
have been generated that develop lung, pancreatic, liver, and intestinal pathologies that
reflect disease in CF patients. Species-specific biology in the processing of CFTR has
demonstrated that pig and mouse ΔF508-CFTR proteins are more effectively processed to
the apical membrane of airway epithelia than human ΔF508-CFTR. The processing …
mutation is ΔF508, and this causes cystic fibrosis (CF). New CF models in the pig and ferret
have been generated that develop lung, pancreatic, liver, and intestinal pathologies that
reflect disease in CF patients. Species-specific biology in the processing of CFTR has
demonstrated that pig and mouse ΔF508-CFTR proteins are more effectively processed to
the apical membrane of airway epithelia than human ΔF508-CFTR. The processing …
