A human SP-C promoter fragment targets alpha 1-proteinase inhibitor gene expression to lung alveolar type II cells in transgenic mice.

A 1.277 kb promoter fragment of the gene encoding one of the lung surfactant proteins, SP-C, was cloned from a human genomic library and characterized using the human alpha 1-proteinase inhibitor (alpha 1PI) gene as reporter. Messenger RNA for human alpha 1PI isolated from a single transgenic mouse line was detected solely in lung tissue. Using immunogold electron microscopy, accumulation of human alpha 1PI was shown unambiguously to occur only in type II pulmonary cells and, in discrete amounts, in the alveolar lining fluid. The protein was secreted and glycosylated showing a molecular weight close to that of plasma-derived human alpha 1PI.