Bio Background |
SFTPB is the pulmonary-associated surfactantprotein B (SPB), an amphipathic surfactant protein essential forlung function and homeostasis after birth. Pulmonary surfactant isa surface-active lipoprotein complex composed of 90% lipids and 10%proteins which include plasma proteins and apolipoproteins SPA, SPB, SPC and SPD. The surfactant is secreted by the alveolar cellsof the lung and maintains the stability of pulmonary tissue byreducing the surface tension of fluids that coat the lung. The SPBenhances the rate of spreading and increases the stability ofsurfactant monolayers in vitro. Multiple mutations in this genehave been identified, which cause pulmonary surfactant metabolismdysfunction type 1, also called pulmonary alveolar proteinosis dueto surfactant protein B deficiency, and are associated with fatalrespiratory distress in the neonatal period. Alternatively splicedtranscript variants encoding the same protein have been identified. |
Bio References |
Baekvad-Hansen, M., et al. Eur. Respir. J. (2010) In press :Kim, W.J., et al. Eur. Respir. J. (2010) In press :Schuurhof, A., et al. Pediatr. Pulmonol. 45(6):608-613(2010)Cho, M.H., et al. Respir. Res. 11, 30 (2010) :Chaiworapongsa, T., et al. J. Matern. Fetal. Neonatal. Med. 21(9):663-670(2008) |