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SERCA Pump Inhibitors Do Not Correct Biosynthetic Arrest of ΔF508 CFTR in Cystic Fibrosis

Deletion of phenylalanine 508 (ΔF508) accounts for nearly 70% of all mutations that occur in the cystic fibrosis transmembrane conductance regulator (CFTR). The ΔF508 mutation is a class II processing mutation that results in very little or no mature CFTR protein reaching the apical membrane and thu...

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Autors principals: Grubb, Barbara R., Gabriel, Sherif E., Mengos, April, Gentzsch, Martina, Randell, Scott H., Van Heeckeren, Anna M., Knowles, Michael R., Drumm, Mitchell L., Riordan, John R., Boucher, Richard C.
Format: Artigo
Idioma:Inglês
Publicat: American Thoracic Society 2006
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2644200/
https://ncbi.nlm.nih.gov/pubmed/16284361
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1165/rcmb.2005-0286OC
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