Article
Residual cftr expression varies with age in cftr(tm1Hgu) cystic fibrosis mice: impact on morphology and physiology.
Pathobiology : journal of immunopathology, molecular and cellular biology - 1 Jan 2000
Larbig M, Jansen S, Dorsch M, Bernhard W, Bellmann B, Dorin J R, Porteous D J, Von Der Hardt H, Steinmetz I, Hedrich H-J, Tuemmler B, Tschernig T
Abstract excerpt
Mouse models for cystic fibrosis (CF) mimic intestinal manifestations of the human disease, but the lung disease phenotypes are lacking in most strains. In this work, the issue was addressed whether aging of the respiratory tract leads to lung pathophysiology in the exon 10 insertional mutant cftr(tm1Hgu) mouse. Weight gain, body weight and life-span of cftr(tm1Hgu) mice were significantly reduced compared with...
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