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Cystic fibrosis: recent structural insights.

Authors
  • Dorwart, Michael
  • Thibodeau, Patrick
  • Thomas, Philip
Type
Published Article
Journal
Journal of Cystic Fibrosis
Publisher
Elsevier
Publication Date
Aug 01, 2004
Volume
3 Suppl 2
Pages
91–94
Identifiers
PMID: 15463935
Source
Medline
License
Unknown

Abstract

Cystic fibrosis (CF) is a debilitating human disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The recently solved crystal structures of the murine CFTR nucleotide binding domain (NBD) provide insight into the molecular basis of several CF-causing mutations. In addition, the NBD structures reveal several unexpected findings that may have implications concerning CFTR function. In this mini-review, we discuss the key structural features of ATP Binding Cassette (ABC) transporter NBDs, as well as highlight how structural information has aided our understanding of the ATP-regulated solute transport cycle.

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