Does lack of Cftr gene lead to developmental abnormalities in the lung?

ABSTRACT

Lung disease is the major cause of death in cystic fibrosis (CF), but the effect of gene mutation on the morphology of the main structural compartments of the lung is poorly understood. We show here, to our knowledge for the first time, a quantitative comparison of the fine pulmonary structures of cftr mutant versus non-cf mice. Pertinent volumes and surface areas were estimated in 10 homozygous cftrm1HGU mutants and 11 non-cf littermates by unbiased stereology at the light microscopic level. Our data did not reveal any statistical differences between group means for any of the 9 parameters considered. In other words, our data do not supply any significant evidence that the lack of the Cftr gene is accompanied by any developmental abnormalities in the lung, at least as far as the parameters studied are concerned.