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The bidirectional relationship between CFTR and lipids.


ABSTRACT: Cystic Fibrosis (CF) is the most common life-shortening genetic disease among Caucasians, resulting from mutations in the gene encoding the Cystic Fibrosis Transmembrane conductance Regulator (CFTR). While work to understand this protein has resulted in new treatment strategies, it is important to emphasize that CFTR exists within a complex lipid bilayer - a concept largely overlooked when performing structural and functional studies. In this review we discuss cellular lipid imbalances in CF, mechanisms by which lipids affect membrane protein activity, and the specific impact of detergents and lipids on CFTR function.

SUBMITTER: Cottrill KA 

PROVIDER: S-EPMC7170930 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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The bidirectional relationship between CFTR and lipids.

Cottrill Kirsten A KA   Farinha Carlos M CM   McCarty Nael A NA  

Communications biology 20200420 1


Cystic Fibrosis (CF) is the most common life-shortening genetic disease among Caucasians, resulting from mutations in the gene encoding the Cystic Fibrosis Transmembrane conductance Regulator (CFTR). While work to understand this protein has resulted in new treatment strategies, it is important to emphasize that CFTR exists within a complex lipid bilayer - a concept largely overlooked when performing structural and functional studies. In this review we discuss cellular lipid imbalances in CF, me  ...[more]

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