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Related Experiment Videos

Structure and function of the CFTR chloride channel

D N Sheppard1, M J Welsh

  • 1Human Genetics Unit, Department of Medicine, University of Edinburgh, Molecular Medicine Centre, Western General Hospital, Edinburgh, United Kingdom.

Physiological Reviews
|January 29, 1999
PubMed
Summary

The cystic fibrosis transmembrane conductance regulator (CFTR) is a unique chloride channel crucial for epithelial salt and fluid transport. Understanding its structure and domain functions aids in comprehending cystic fibrosis and related disorders.

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Area of Science:

  • Biophysics
  • Molecular Biology
  • Physiology

Background:

  • The cystic fibrosis transmembrane conductance regulator (CFTR) is an ABC transporter family member forming a novel chloride channel.
  • CFTR is vital for transepithelial salt and liquid movement in epithelia.
  • CFTR dysfunction leads to the genetic disorder cystic fibrosis.

Purpose of the Study:

  • To review the structure and function of the CFTR chloride channel.
  • To elucidate the roles of CFTR's distinct domains in channel activity.
  • To connect CFTR structure-function insights to its physiological roles and disease mechanisms.

Main Methods:

  • Review of existing literature on CFTR structure and function.
  • Analysis of the contributions of CFTR's five domains (MSDs, NBDs, R domain) to channel operation.

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  • Integration of structural and functional data to explain channel gating and regulation.

    • Main Results:

    • CFTR comprises two membrane-spanning domains (MSDs), two nucleotide-binding domains (NBDs), and a regulatory (R) domain.
    • MSDs form the ion pore.
    • R domain phosphorylation regulates activity, while NBDs control gating via ATP hydrolysis.

    Conclusions:

    • CFTR's modular structure dictates its function as a regulated chloride channel.
    • Understanding CFTR's molecular mechanisms is key to addressing cystic fibrosis.
    • CFTR's unique properties offer insights into the broader ABC transporter superfamily.