Prosurfactant Protein B è un gene codificato dal simbolo SFTPB. Comunemente indicato anche come: 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 ha una massa di 42.12kDa, una lunghezza di amminoacidi di 381, ed è implicato nella malattia: Pulmonary surfactant metabolism dysfunction 1; Respiratory distress syndrome in premature infants.
Offriamo 3 anticorpi contro Prosurfactant Protein B, allevati nel Coniglio, che sono adatti per WB, IHC e ELISA con campioni derivati da Umano.
Informazioni su geni e proteine
Riepilogo 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.
Sommario di 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.
Coinvolgimento nella malattia
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'.
Posizione cellulare
Secreted > Extracellular space > Surface film.
