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Cystic fibrosis 

Cystic fibrosis
Cystic fibrosis

Andrew Bush

and Caroline Elston



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.

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date: 24 September 2021

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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