Cohen J, Lundblad L, Bates J, Levitzky M, Larson J. The "Goldilocks effect" in cystic fibrosis: identification of a lung phenotype in the cftr knockout and heterozygous mouse. BMC Genet. 2004;5:21 pubmed
..Distinct phenotypes were demonstrated in each genotype, cftr-/-, cftr +/-, and cftr+/+. These results are consistent with a developmental role for CFTR in the lung. ..
Cohen J, Larson J. Pathophysiologic consequences following inhibition of a CFTR-dependent developmental cascade in the lung. BMC Dev Biol. 2005;5:2 pubmed
..In the lung CFTR is part of a developmental cascade for normal secretory cell differentiation. Absence of CFTR results in a constitutive inflammatory process that is involved in some aspects of CF pathophysiology. ..