| First Author | Weaver TE | Year | 1999 |
| Journal | Biol Neonate | Volume | 76 Suppl 1 |
| Pages | 15-8 | PubMed ID | 10393388 |
| Mgi Jnum | J:56178 | Mgi Id | MGI:1340336 |
| Doi | 10.1159/000047041 | Citation | Weaver TE, et al. (1999) Use of knockout mice to study surfactant protein structure and function. Biol Neonate 76 Suppl 1:15-8 |
| abstractText | Pulmonary surfactant protein B (SP-B) is a 79 amino acid peptide that is intimately associated with surfactant phospholipids in the alveolar airspace. Mutations of the SP-B gene that result in complete absence of SP-B are invariably fatal in the neonatal period. The pathology associated with SP-B deficiency suggests that SP-B plays a critical role in integrating the synthesis, assembly and metabolism of the surfactant complex. A strategy is described to elucidate the role of SP-B in surfactant homeostasis by characterizing the pathophysiology associated with cell specific expression of SP-B constructs in vivo. Human SP-B constructs, under control of lung cell-specific promoters, were expressed in SP-B knockout mice in order to achieve expression of the human transgene in a null background. The effect of transgene expression on lung structure and function was assessed by biochemical, morphological and physiological analyses of the surfactant system in fetal and postnatal offspring. |
