Pulmonary surfactant is a mixture of phospholipids and protein, which is surface-active and secreted by type II epithelial cells into the alveolar space. There are four proteins present in the surfactant namely, SP (surfactant protein)-A, SP-B, SP-C or SFTPC and SP-D. SFTPC is a hydrophobic protein, which is produced as a proprotein and undergoes post-translational modification to have its NH2 and COOH propeptides removed. The mature active protein has a molecular weight of 3.7kDa. The proprotein is composed of 197 amino acids and is produced exclusively by alveolar type 2 (AT2) cells. SFTPC contains BRICHOS domain, which is suggested to prevent the aggregation of the peptide and regulate proteolytic processing of SFTPC proprotein. This gene is located on human chromosome 8p21.
Synonyms: Anti-BRICD6; Anti-PSP-C; Anti-SFTP2; Anti-SMDP2; Anti-SP-C
Storage: -20C
Application: Anti-SFTPC antibody produced in rabbit has been used in immunohistochemistry (IHC) and immunofluorescence (IF).
Biochem Physiol Actions: SFTPC (surfactant protein C) stabilizes the pulmonary surfactant film and aids in the adsorption of phospholipids on the surfactant monolayer. This way it regulates the metabolism and dynamics of the phospholipids constituting pulmonary surfactant. Mutations in this gene are associated with interstitial lung disease (ILD) in infants. Mutations in SFTPC can lead to various disorders such as, chronic pneumonitis in infants, and usual or desquamative interstitial pneumonia and idiopathic pulmonary fibrosis in adults. G100S mutation in the BRICHOS domain of SFTPC proprotein, leads to increased endoplasmic reticulum (ER) stress and cell apoptosis. Therefore, G100S mutation in this gene is involved in the pathogenesis of familial pulmonary fibrosis in Japanese population. Mutations in SFTPC gene also result in combined pulmonary fibrosis and emphysema (CPFE) syndrome, which is characterized by emphysema, inflammation and fibrosis.
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