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Cystic fibrosis 

Cystic fibrosis
Chapter:
Cystic fibrosis
Author(s):

Andrew Bush

and Caroline Elston

DOI:
10.1093/med/9780199204854.003.1810_update_003

Update:

Infection—increased recognition of the importance of lung infection with Mycobacterium abscessus.

Future prospects—new animal models that more precisely replicate human disease; the highly efficacious use of Ivacaftor in patients with the G551D mutation, who comprise 4–5% of patients with CF.

Updated on 28 Aug 2014. The previous version of this content can be found here.
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date: 17 October 2017

Cystic Fibrosis (CF) is a recessively inherited disease caused by mutations in the cystic fibrosis gene, located on the long arm of chromosome 7, which codes for a membrane protein—the cystic fibrosis transmembrane regulator protein (CFTR)—that is a chloride channel. Around 1300 CF mutations have been identified, with the ...

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