ABCMdb

PMID: 20044437

Hampton TH, Stanton BA
A novel approach to analyze gene expression data demonstrates that the DeltaF508 mutation in CFTR downregulates the antigen presentation pathway.
Am J Physiol Lung Cell Mol Physiol. 2010 Apr;298(4):L473-82. Epub 2009 Dec 31., [PubMed]

Sentences

No. Mutations Sentence Comment

33 ABCC7 p.Trp1282* The lack of similar findings among these studies may be due to experimental differences, including the type of tissue examined (i.e., nasal brushings, stable clones of airway epithelial cells, airway epithelial cells in primary culture, and stable bronchial epithelial cell lines), the genotype of the cells used (i.e., ⌬F508/⌬F508 vs. ⌬F508/W1282X), and because no two studies used the same statistical approach to identify differentially expressed genes. Login to comment 49 ABCC7 p.Trp1282* Virella-Lowell et al. (44) used immortalized bronchial epithelial cells expressing the ⌬F508/ W1282X mutation (IB3-1 cells isolated from a single individual) and IB3-1 cells rescued with wild-type (wt)-CFTR (S9). Login to comment 66 ABCC7 p.Trp1282* Summary of the experimental designs used by the original authors CF Genotype Tissue N Affymetrix Platform Reference ⌬F508/W1282X Isogenic bronchial cells (IB3-1 and S9) 1/1 U95Av2 Virella-Lowell et al. (44) ⌬F508/⌬F508 Tracheal and bronchial cells in primary culture 10/10 HGU133A Zabner et al. (50) ⌬F508/⌬F508 Nasal brushings 12/11* HGU-133A,B Wright et al. (47) ⌬F508/⌬F508 Fetal tracheal cells (CFT-2 and NT-1) 1/1 HGU-133Plus2 Verhaeghe et al. (43) Most studies compared nonisogenic samples from cystic fibrosis (CF) and non-CF patients bearing a homozygous ⌬F508 mutation. Login to comment