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

Lung disease is the major cause of death in cystic fibrosis (CF), bur the e ffect of gene mutation on the morphology of the main structural compartment s, of the lung is poorly understood.We show here to our knowledge for the f irst time , a quantitative comparison of the fine pulmonary structures of c ftr mutant versus non-cf mice. Pertinent volumes and surface areas were est imated in 10 homozygous cftr(mlHGU) mutnnmutants and 11 non-cf littermates by unbiased stereology at the light microscopic level. Our data did not rev eal any statistical differences between group, means for any of the 9 param etres considered. In other words, our data do not supply any significant ev idence that the lack of the Cftr gene is accompanied by any developmental a bnormalities in the lung, at least as far as the parametres studied are con cerned.