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Journal article

Differential thermostability and response to cystic fibrosis transmembrane conductance regulator (CFTR) potentiators of human and mouse F508del-CFTR

Abstract:

Cross-species comparative studies have highlighted differences between human and mouse cystic fibrosis transmembrane conductance regulator (CFTR), the epithelial Cl− channel defective in cystic fibrosis (CF). Here, we compare the impact of the most common CF mutation F508del on the function of human and mouse CFTR heterologously expressed in mammalian cells and their response to CFTR modulators using the iodide efflux and patch-clamp techniques. Once delivered to the plasma membrane, human F5...

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Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.1152/ajplung.00034.2019

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Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Pharmacology
Department:
Unknown
Role:
Author
Publisher:
American Physiological Society
Journal:
AJP - Lung Cellular and Molecular Physiology More from this journal
Volume:
317
Issue:
1
Pages:
L71-L86
Publication date:
2019-04-10
Acceptance date:
2019-04-04
DOI:
EISSN:
1522-1504
ISSN:
1040-0605
Language:
English
Keywords:
Pubs id:
pubs:995053
UUID:
uuid:7d504c40-a77e-4e90-8817-d4edb0cbe2a8
Local pid:
pubs:995053
Source identifiers:
995053
Deposit date:
2019-04-30

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