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About CF » Genetics and cell biology of CFTR » CFTR structure and regulation

CFTR structure and regulation

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The CFTR protein is a member of the ABC transporter superfamily of proteins that use the energy from nucleotide hydrolysis to transport molecules across the membrane.1 CFTR is composed of five functional domains.1,2

TMDs or ‘transmembrane domains’:
Around 19% of CFTR is composed of TMD1 and TMD2, which form the channel pore allowing transport of chloride ions across the membrane.3

NBDs or ‘nucleotide-binding domains’:
These domains bind the nucleotide molecule ATP (a vehicle of chemical energy). Opening and closing of the channel (or ‘gating’) requires ATP to bind to these domains.4

Regulatory (‘R’) domain:
The R domain regulates channel activity and can be considered to be the ‘trigger’ governing whether the channel opens or closes, to activate the channel.1,2,4

Did you know?
Many CF-causing mutations occur in NBD1, including F508del, while fewer occur in NBD2.

The CFTR protein is composed of 1480 amino acids arranged into five functional domains that play a vital role in the regulation of chloride and bicarbonate ions across the membrane.1
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References
  1. Patrick AE et al. Front Pharmacol 2012;3:162
  2. Rowe SM & Miller S. N Engl J Med 2005;352:1992–2001
  3. Riordan JR. Annu Rev Physiol 2005;67:701–18
  4. Paola Vergani et al. Nature 2005;433:876–80