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Cystic fibrosis in 2016: considerable progress, but much more to do

Jane C Davies1

  • 1Imperial College London and Royal Brompton & Harefield Foundation Trust, London SW3 6LR, UK.

The Lancet. Respiratory Medicine
|November 29, 2016
PubMed
Abstract

No abstract available in PubMed .

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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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