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About CF » Genetics and cell biology of CFTR » CFTR structure and regulation (cont.)

CFTR structure and regulation (cont.)

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Opening of the CFTR channel requires two main changes to occur in the structure of the protein:1-3

  1. Phosphate molecules bind to the regulatory domain (R) in a process called phosphorylation
  2. Molecules called nucleotides (ATP, ADP) bind to the nucleotide-binding domain (NBD)

These events bring about conformational changes in the structure of CFTR that allow the channel to open.

As this is reversible, CFTR can open and close at varying frequencies – also known as the ‘open probability‘.

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References
1. Bush A et al. Pediatr Pulmonol 2005;39:289–91
2. MacDonald KD et al. Paediatr Drugs 2007;9:1–10
3. Hennager DJ et al. Proc Natl Acad Sci USA 2001;98:3594–9