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Endocytic trafficking of CFTR in health and disease Ameen N; Silvis M; Bradbury NAJ Cyst Fibros 2007[Jan]; 6 (1): 1-14The cystic fibrosis transmembrane conductance regulator (CFTR) is a Cl-selective anion channel expressed in epithelial tissues. Mutations in CFTR lead to the genetic disease cystic fibrosis (CF). Within each epithelial cell, CFTR interacts with a large number of transient macromolecular complexes, many of which are involved in the trafficking and targeting of CFTR. Understanding how these complexes regulate the trafficking and fate of CFTR, provides a singular insight not only into the patho-physiology of cystic fibrosis, but also provides potential drug targets to help cure this debilitating disease.|Animals[MESH]|Cystic Fibrosis Transmembrane Conductance Regulator/genetics/metabolism/*physiology[MESH]|Cystic Fibrosis/*genetics/*physiopathology[MESH]|Endocytosis/*physiology[MESH]|Humans[MESH]|Sequence Alignment[MESH]|Structure-Activity Relationship[MESH]|Transport Vesicles/physiology[MESH] |
