PMID: 19168701

Yu H, Zeidan YH, Wu BX, Jenkins RW, Flotte TR, Hannun YA, Virella-Lowell I
Defective acid sphingomyelinase pathway with Pseudomonas aeruginosa infection in cystic fibrosis.
Am J Respir Cell Mol Biol. 2009 Sep;41(3):367-75. Epub 2009 Jan 23., [PubMed]
Sentences
No. Mutations Sentence Comment
34 ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 19168701:34:80
status: NEW
view ABCC7 p.Trp1282* details
 MATERIALS AND METHODS Cell Lines The IB3-1 cell line, the CFTR-defective (DF508/W1282X) immortalized bronchial epithelial cell line from a patient with CF (21), and S9 cell line, a stable IB3-1 subclone transduced with the recombinant AAV-CFTR vector, which expresses a functional Cl-channel (22-24), were used in this study. Login to comment 79 ABCC7 p.Ser489*
X
ABCC7 p.Ser489* 19168701:79:159
status: NEW
view ABCC7 p.Ser489* details
 Animal Experiments The CFTR KO mice are Cftrtm1Unc-TgN(FABPCFTR) (fatty acid-binding protein [FABP]-CFTR) mice that have a stop codon in the murine CFTR gene (S489X) but also express human CFTR in the gut epithelium due to transgenic introduction of CFTR under the control of the FABP promoter (30). Login to comment