SURFACTANT PROTEIN C (SP-C, Pro, SFTPC, SFTP2, PSP-C, SMDP2)

This gene encodes the pulmonary-associated surfactant protein C (SPC), an extremely hydrophobic 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. Multiple mutations in this gene have been identified, which cause pulmonary surfactant metabolism dysfunction type 2, also called pulmonary alveolar proteinosis due to surfactant protein C deficiency, and are associated with interstitial lung disease in older infants, children, and adults. Alternatively spliced transcript variants encoding different protein isoforms have been identified. \n\nThere are four surfactant-specific proteins, designated surfactant protein A (SP-A), SP-B, SP-C and SP-D respectively. SP-A and SP-D are hydrophilic surfactant proteins and are members of the collectin family. SP-B and SP-C are hydrophobic surfactant proteins and may be the most appropriate indicators for the evolutionary origin of surfactant. SP-C is a 34-35 amino acid peptide, of 4kD that is proteolytically processed from a 21kD precursor protein. SP-C is initiated early in the embryogenic period of lung formation, where SP-C transcripts are detected uniformly in epithelial cells lining the primitive airways. During lung development SP-C expression is decreased in cells of the proximal conducting portion of the lung. Ultimately SP-C is expressed selectively in type II epithelial cells in the alveolus of the lung. SP-C is secreted into the airspace where it enhances the spreading and stability of surfactant phospholipids in the alveolus. SP-C plays an important role in the spreading and stabilization of phospholipid films in the alveolus. SP-C is essential for air-breathing in mammals and is therefore largely conserved. Deficiency of SP-C and other surfactant components is associated with respiratory distress syndrome (RDS) in premature infants and adults with respiratory distress syndrome (ARDS). \n\n\nApplications:\nWestern Blot: 1:500-1:2000\nELISA: 1:10000\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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