Prosurfactant Protein B est un gène codé par le symbole SFTPB. Communément appelé aussi: Pulmonary surfactant-associated protein B; SP-B; 18 kDa pulmonary-surfactant protein; 6 kDa protein; Pulmonary surfactant-associated proteolipid SPL(Phe); SFTPB; SFTP3. Prosurfactant Protein B a une masse de 42.12kDa, une longueur d'acide aminé de 381, et est impliqué dans les maladies: Pulmonary surfactant metabolism dysfunction 1; Respiratory distress syndrome in premature infants.
Nous proposons 3 des anticorps contre Prosurfactant Protein B, élevé dans Lapin, qui sont appropriés pour le WB, IHC et ELISA avec des échantillons dérivés de Humain.
Informations sur les Gènes et les Protéines
Résumé UniProt
Pulmonary surfactant-associated proteins promote alveolar stability by lowering the surface tension at the air-liquid interface in the peripheral air spaces. SP-B increases the collapse pressure of palmitic acid to nearly 70 millinewtons per meter.
Résumé Entrez
This gene encodes the pulmonary-associated surfactant protein B (SPB), an amphipathic surfactant protein essential for lung function and homeostasis after birth. Pulmonary surfactant is a 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 cells of the lung and maintains the stability of pulmonary tissue by reducing the surface tension of fluids that coat the lung. The SPB enhances the rate of spreading and increases the stability of surfactant monolayers in vitro. Multiple mutations in this gene have been identified, which cause pulmonary surfactant metabolism dysfunction type 1, also called pulmonary alveolar proteinosis due to surfactant protein B deficiency, and are associated with fatal respiratory distress in the neonatal period. Alternatively spliced transcript variants encoding the same protein have been identified.
Implication dans la maladie
Pulmonary surfactant metabolism dysfunction 1: A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.
Respiratory distress syndrome in premature infants: A lung disease affecting usually premature newborn infants. It is characterized by deficient gas exchange, diffuse atelectasis, high-permeability lung edema and fibrin-rich alveolar deposits called 'hyaline membranes'.
Localisation cellulaire
Secreted > Extracellular space > Surface film.
