|Other Names||Pulmonary surfactant-associated protein C, SP-C, Pulmonary surfactant-associated proteolipid SPL(Val), SP5, SFTPC, SFTP2|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Pulmonary surfactant associated proteins promote alveolar stability by lowering the surface tension at the air- liquid interface in the peripheral air spaces.|
|Cellular Location||Secreted, extracellular space, surface film.|
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This gene encodes the pulmonary-associated surfactantprotein C (SPC), an extremely hydrophobic surfactant proteinessential for lung function and homeostasis after birth. Pulmonarysurfactant is a surface-active lipoprotein complex composed of 90%lipids and 10% proteins which include plasma proteins andapolipoproteins SPA, SPB, SPC and SPD. The surfactant is secretedby the alveolar cells of the lung and maintains the stability ofpulmonary tissue by reducing the surface tension of fluids thatcoat the lung. Multiple mutations in this gene have beenidentified, which cause pulmonary surfactant metabolism dysfunctiontype 2, also called pulmonary alveolar proteinosis due tosurfactant protein C deficiency, and are associated withinterstitial lung disease in older infants, children, and adults.Alternatively spliced transcript variants encoding differentprotein isoforms have been identified.
Wambach, J.A., et al. Pediatr. Res. 68(3):216-220(2010)Schuurhof, A., et al. Pediatr. Pulmonol. 45(6):608-613(2010)Thouvenin, G., et al. Arch. Dis. Child. 95(6):449-454(2010)Crossno, P.F., et al. Chest 137(4):969-973(2010)Davila, S., et al. Genes Immun. 11(3):232-238(2010)
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