A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.

The most prevalent mutation (delta F508) in cystic fibrosis patients inhibits maturation and transfer to the plasma membrane of the mutant cystic fibrosis transmembrane conductance regulator (CFTR). We have analyzed the properties of a delta F508 CFTR mouse model, which we described recently. We sho...

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Udgivet i:J Clin Invest
Main Authors: French, P J, van Doorninck, J H, Peters, R H, Verbeek, E, Ameen, N A, Marino, C R, de Jonge, H R, Bijman, J, Scholte, B J
Format: Artigo
Sprog:Inglês
Udgivet: American Society for Clinical Investigation 1996
Fag:
Research Article
Online adgang:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC507556/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/8823295/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI118917
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