This gene encodes the pulmonary-associated surfactantnprotein C (SPC), an extremely hydrophobic surfactant proteinnessential for lung function and homeostasis after birth. Pulmonarynsurfactant is a surface-active lipoprotein complex composed of 90%nlipids and 10% proteins which include plasma proteins andnapolipoproteins SPA, SPB, SPC and SPD. The surfactant is secretednby the alveolar cells of the lung and maintains the stability ofnpulmonary tissue by reducing the surface tension of fluids thatncoat the lung. Multiple mutations in this gene have beennidentified, which cause pulmonary surfactant metabolism dysfunctionntype 2, also called pulmonary alveolar proteinosis due tonsurfactant protein C deficiency, and are associated withninterstitial lung disease in older infants, children, and adults.nAlternatively spliced transcript variants encoding differentnprotein isoforms have been identified.
应用类型
WB,IHC
免疫原
This SFTPC antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 144-173 amino acids from the C-terminal region of human SFTPC.
