Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation

Most cases of cystic fibrosis (CF) are caused by mutations that block the biosynthetic maturation of the CF gene product, the CF transmembrane conductance regulator (CFTR) chloride channel. CFTR-processing mutants fail to escape the endoplasmic reticulum and are rapidly degraded. Current efforts to...

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Bibliographische Detailangaben
Hauptverfasser: Cormet-Boyaka, Estelle, Jablonsky, Michael, Naren, Anjaparavanda P., Jackson, Patricia L., Muccio, Donald D., Kirk, Kevin L.
Format: Artigo
Sprache:Inglês
Veröffentlicht: National Academy of Sciences 2004
Schlagworte:
Biological Sciences
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC419584/
https://ncbi.nlm.nih.gov/pubmed/15141088
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0400459101
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