ABCMdb

PMID: 20935229

Rubenstein RC, Lockwood SR, Lide E, Bauer R, Suaud L, Grumbach Y
Regulation of endogenous ENaC functional expression by CFTR and DeltaF508-CFTR in airway epithelial cells.
Am J Physiol Lung Cell Mol Physiol. 2011 Jan;300(1):L88-L101. Epub 2010 Oct 8., [PubMed]

Sentences

No. Mutations Sentence Comment

300 ABCC7 p.Gly551Asp In fact, recent data by Van Goor and colleagues (64) suggest that potentiation (or enhancement) of the function of G551D CFTR with the novel small molecule VX-770 decreases amiloride-inhibited transepithelial potential difference in human bronchial epithelial cells within a few minutes. Login to comment 305 ABCC7 p.Ile148Thr Coexpression of CFTRs with appropriate intracellular trafficking [wt (67) and I148T (60)] decrease ENaC functional, surface, and/or whole oocyte expression compared with oocytes expressing ENaC alone in the absence of specific treatments to activate CFTR (i.e., without forskolin/IBMX stimulation). Login to comment 306 ABCC7 p.Asn1303Lys In contrast, coexpression of mutant CFTRs with defective trafficking [⌬F508 (67) and N1303K (59)] did not alter ENaC functional, surface, and/or whole oocyte expression when under similar, nonstimulated conditions. Login to comment 320 ABCC7 p.Gly551Asp (64) discussed above where inhibition of VX-770 potentiated G551D CFTR-increased amiloride-sensitive transepithelial potential difference, we do not feel that these data are contradictory. Login to comment