Ouabain mimics low temperature rescue of F508del-CFTR in cystic fibrosis epithelial cells

• Главные авторы: Donglei eZhang (Автор), Fabiana eCiciriello (Автор), Suzana eAnjos (Автор), Annamaria eCarissimo (Автор), Jie eLiao (Автор), Graeme eCarlile (Автор), Haouaria eBalghi (Автор), Renaud eRobert (Автор), Alberto eLuini (Автор), John eHanrahan (Автор), David eThomas (Автор)
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• Опубликовано: Frontiers Media S.A., 2012-10-01T00:00:00Z.
• Предметы: article
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ABSTRACT Most cases of cystic fibrosis (CF) are caused by the deletion of a single phenylalanine residue at position 508 of the cystic fibrosis transmembrane conductance regulator (CFTR). The mutant F508del-CFTR is retained in the endoplasmic reticulum and degraded, but can be induced by low tempera...