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

• Main Authors: Donglei eZhang (Author), Fabiana eCiciriello (Author), Suzana eAnjos (Author), Annamaria eCarissimo (Author), Jie eLiao (Author), Graeme eCarlile (Author), Haouaria eBalghi (Author), Renaud eRobert (Author), Alberto eLuini (Author), John eHanrahan (Author), David eThomas (Author)
• Format: Book
• Published: Frontiers Media S.A., 2012-10-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...