ABCMdb

PMID: 16763223

Velsor LW, Kariya C, Kachadourian R, Day BJ
Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
Am J Respir Cell Mol Biol. 2006 Nov;35(5):579-86. Epub 2006 Jun 8., [PubMed]

Sentences

No. Mutations Sentence Comment

40 ABCC7 p.Ser489* Animal Care and Use This study compared the mitochondrial consequences of the CFTR mutation from two congenic CFTR KO strains (S489X and FABP breeding stock were kind gifts from Dr. Anna van Heeckeren, Case Western Reserve University, Cleveland, OH) with C57B6 control mice. Login to comment 41 ABCC7 p.Ser489* In the S489X congenic C57B6 strain, a mutation creates a stop codon and produces a truncated CFTR protein (19). Login to comment 43 ABCC7 p.Ser489* To avoid this problem, S489X mice are maintained on a liquid diet (Peptamen; Nestle, Glendale, CA). Login to comment 45 ABCC7 p.Ser489* With the exception of the liquid diet for the S489X mice, all mice were provided solid mouse chow and autoclaved tap water ad libitum. Login to comment 49 ABCC7 p.Trp1282* These cells possess heterozygous mutations (⌬F508 and W1282X) for the CFTR gene. Login to comment 138 ABCC7 p.Ser489* In the S489X CFTR KO mice on a liquid diet, mitochondrial GSH concentrations were decreased 43% compared with controls. Login to comment 148 ABCC7 p.Ser489* Mitochondria isolated from the lungs of S489X and FABP CFTR KO mice displayed significantly lower levels of GSH than mitochondria from control mice. Login to comment 206 ABCC7 p.Ser489* Both S489X and FABP CFTR KO mice had significantly lower mitochondrial GSH concentrations than control mice. Login to comment 208 ABCC7 p.Ser489* Between the two CFTR KO mouse strains, however, the FABP GSH levels were considerably lower than the levels in the S489X mice. Login to comment 209 ABCC7 p.Ser489* The S489X mutation generates a stop codon and produces a truncated protein (19). Login to comment 210 ABCC7 p.Ser489* The S489X CFTR KO mouse must be maintained on a liquid diet to prevent fatal bowel obstructions. Login to comment