ABCMdb

PMID: 18790990

Manson ME, Corey DA, White NM, Kelley TJ
cAMP-mediated regulation of cholesterol accumulation in cystic fibrosis and Niemann-Pick type C cells.
Am J Physiol Lung Cell Mol Physiol. 2008 Nov;295(5):L809-19. Epub 2008 Sep 12., [PubMed]

Sentences

No. Mutations Sentence Comment

58 ABCC7 p.Trp1282* IB3-1 cells (⌬F508/W1282X) and S9 cells (IB3-1 cell stably transfected with the full-length wild-type CFTR as controls) were developed by Pamela L. Zeitlin (Johns Hopkins University, Baltimore, MD). Login to comment 61 ABCC7 p.Trp1282* IB3-1 cells (⌬F508/W1282X) and S9 cells (IB3-1 cell stably transfected with the full-length wild-type CFTR as controls) were developed by Pamela L. Zeitlin (Johns Hopkins University, Baltimore, MD). Login to comment 121 ABCC7 p.Ser489* To verify results in an in vivo model, we excised nasal epithelium from S489X Cftr-/- mice and assayed for pCREB content compared with nasal epithelium from age-matched wild-type mice. Login to comment 124 ABCC7 p.Ser489* To verify results in an in vivo model, we excised nasal epithelium from S489X Cftr-/- mice and assayed for pCREB content compared with nasal epithelium from age-matched wild-type mice. Login to comment 207 ABCC7 p.Gly551Asp The expression of the inactive but properly trafficked G551D CFTR mutant has no effect on cholesterol transport. Login to comment 210 ABCC7 p.Gly551Asp The expression of the inactive but properly trafficked G551D CFTR mutant has no effect on cholesterol transport. Login to comment 216 ABCC7 p.Trp1282* We have shown that Cftr-/- mice exhibit cholesterol processing defects (41), and we have demonstrated that cholesterol processing in IB3 (W1282X/⌬F508) is restored in CFTR-corrected IB3 cells (S9 cells) (40). Login to comment 219 ABCC7 p.Gly551Asp ABCC7 p.Trp1282* We have shown that Cftr-/- mice exhibit cholesterol processing defects (41), and we have demonstrated that cholesterol processing in IB3 (W1282X/⌬F508) is restored in CFTR-corrected IB3 cells (S9 cells) (40). Login to comment 222 ABCC7 p.Gly551Asp Expression of G551D CFTR in CHO cells is unlikely to Fig. 6. Correction of cholesterol transport in CF model IB3 cells with Rp-cAMPS. Login to comment