Anti-Prosurfactant Protein C Antibody

Pulmonary surfactant Protein C (SP-C) is one of 4 distinct surfactant proteins; SP-A, SP-B, SP-C and SP-D. SP-C is produced by proteolytic cleavage of a larger precursor known as Prosurfactant Protein C which is expressed exclusively in the lungs and widely used as a marker for alveolar type 2 epithelial cells. 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.