IMPORTANT INFORMATION

  • CFTR.info is an online educational resource on the genetics of cystic fibrosis (CF) and the role of CF transmembrane conductance regulator (CFTR) in normal human physiology as well as in CF.
  • The information contained herein is intended for healthcare professionals working within the European Union (EU).
  • The content of this website has been developed independently by a group of CF healthcare professionals and is for general information purposes only.
  • All of the information about CFTR and CFTR modulators has been obtained from referenced sources and is in the public domain.
  • This website is not intended to promote or advocate a particular course of treatment or any specific drug, whether licensed or unlicensed, nor is it intended to imply or suggest that any drug be used for any purpose other than that which for which it is licensed in the EU.

ABBREVIATED TERMS & CONDITIONS

  • While we endeavour to keep the information up to date and correct, we make no representations or warranties of any kind, express or implied, about the completeness, accuracy, reliability, suitability or availability with respect to the website or the information and related graphics, or products and treatments, contained on the website for any purpose. Any reliance you place on such information is therefore strictly at your own risk.
  • In no event will we be liable for any loss or damage including without limitation, indirect or consequential loss or damage, or any loss or damage whatsoever arising from, or in connection with, the use of this website.
  • Through this website you are able to link to other websites which are not under our control. We have no control over the nature, content and availability of those sites. The inclusion of any links does not necessarily imply a recommendation or endorse the views expressed within them.
  • Every effort is made to keep the website up and running smoothly. However, we take no responsibility for, and will not be liable for, the website being temporarily unavailable due to technical issues beyond our control.

Please see the full Terms & Conditions before using this website.

 

I have read and understood the above

About CF » Genetics and cell biology of CFTR » CFTR journey

CFTR journey

Click on the tabs to move through the sequence.

The entire process of CFTR protein synthesis can be broken down into five main steps:1-4

1. Transcription

2. Translation and protein folding

3. Post-translational modification

4. Protein trafficking

5. Surface expression

Transcription (part 1)

Under normal circumstances the CFTR gene is tightly packed into a supercoil of DNA that forms the chromosome.

 

 

 

 

 

Transcription (part 2)

CFTR gene expression is initiated by cell signals (e.g. hormones) that instruct the chromosome to unravel and reveal the CFTR gene. A portable copy of the gene is made in the form of messenger RNA (mRNA) – this process is called transcription.

Transcription (part 3)

The mRNA strand passes through the nuclear pore into cytoplasm to begin the process of translation.

Translation and protein folding

Ribosomes ‘read’ and translate the mRNA sequence into a polypeptide chain of amino acids (i.e. the immature CFTR protein). This is called translation. Upon reading the final three-letter signal or ‘STOP codon’, the ribosome releases the fully extended CFTR polypeptide, which ‘folds’ to form an immature CFTR channel.

Post-translational modification

After protein translation, the immature CFTR protein undergoes post-translational modification in Golgi bodies. During this process the unique 3-D structure of the CFTR protein continues to develop through a series of chemical modifications. This culminates with the channel being packaged in a vesicle for transport to the cell surface.

Protein trafficking

After post-translational modification, CFTR is packaged in vesicles for transport to the cell surface. This is called protein trafficking.

Surface expression

Finally, the vesicle carrying CFTR protein fuses with the membrane, allowing surface expression of CFTR. The CFTR can immediately begin functioning as an ion channel.

References

1. Rogan MP et al. Chest 2011;139:1480–90
2. Rowe S et al. N Engl J Med 2005;352:1992–2001
3. Ratjen F. Respir Care 2009;54:595–605
4. McCarthy V et al. Pediatr Pulmonol 2005;40:1–8