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Endocytic trafficking of CFTR in health and disease.


ABSTRACT: The 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.

SUBMITTER: Ameen N 

PROVIDER: S-EPMC1964799 | biostudies-literature | 2007 Jan

REPOSITORIES: biostudies-literature

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Endocytic trafficking of CFTR in health and disease.

Ameen Nadia N   Silvis Mark M   Bradbury Neil A NA  

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 20061113 1


The 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-phy  ...[more]

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