ABCMdb

PMID: 11116277

Roomans GM
Pharmacological treatment of the ion transport defect in cystic fibrosis.
Expert Opin Investig Drugs. 2001 Jan;10(1):1-19., [PubMed]

Sentences

No. Mutations Sentence Comment

125 ABCC7 p.Gly551Asp Yang et al. found that high concentrations of IBMX (4 mM) could induce chloride secretion in cells with the G551D-CFTR mutation, but only in 20% of cells with the ∆F508 mutation [46]. Login to comment 182 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp On the other hand, genistein activates the trafficking-competent G551D-CFTR mutant without pretreatment with 4PBA [34]. Login to comment 194 ABCC7 p.Gly551Asp Also, a variety of other phosphatase inhibitors, such as levamisole and bromotetramisole [98-100], cyclosporin A and deltamethrin [101] have been shown to activate wild-type and mutated (G551D and ∆F508) CFTR. Login to comment 196 ABCC7 p.Gly551Asp It has also been claimed that class III phosphodiesterase inhibitors such as milrinone could activate ∆F508-CFTR [102], but according to a recent study on nasal epithelia of ∆F508 or G551D mice, as well as on nasal epithelia of non-CF controls and CF patients, milrinone is without significant effect on chloride efflux in these tissues [103]. Login to comment 199 ABCC7 p.Pro574His A substituted benzimidazolone, NS-004, was shown to activate wild-type CFTR and ∆F508-CFTR chloride channels in Xenopus oocytes and Vero cells expressing these channels [105] and also restored near-normal CFTR activity in cells expressing the P574H-CFTR channel (a mild mutation of CF) [106]. Login to comment 219 ABCC7 p.Arg553* ABCC7 p.Gly542* 5.6 Gentamicin The aminoglycoside antibiotic gentamicin has been shown to suppress two premature stop mutations (G542X and R553X) [114]. Login to comment 410 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp 34. ILLEK B, ZHANG L, LEWIS NC, MOSS RB, DONG JY, FISCHER H: Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein. Am. J. Physiol. Login to comment