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Pediatric Lung Disease
Published in Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley, Diagnostic Pulmonary Pathology, 2008
The surfactant dysfunction disorders are a group of lung diseases occurring predominantly in infants and children that are caused by mutations in three proteins affecting surfactant metabolism: surfactant protein B (SP-B) (SFTPB gene on chromosome 2p12-p11.2), surfactant protein C (SP-C) (SFTPC gene on chromosome 8p21), and the ATP-binding cassette subfamily A member 3 protein (ABCA3 gene on chromosome 16p13.3) (34–36). Mutations in SFTPB and ABCA3 genes have an autosomal recessive pattern of inheritance, while SFTPC mutations are autosomal dominant, in some cases manifesting as chronic lung disease in successive generations (37–39). It should be noted that a subset of cases with typical histologic features of the genetic surfactant disorders have no mutations detected in these three genes, suggesting the presence of other currently unknown causative genes.
MiR-629-3p-induced downregulation of SFTPC promotes cell proliferation and predicts poor survival in lung adenocarcinoma
Published in Artificial Cells, Nanomedicine, and Biotechnology, 2019
Bin Li, Yu-Qi Meng, Zheng Li, Ci Yin, Jun-Ping Lin, Duo-Jie Zhu, Shao-Bo Zhang
Pulmonary surfactant is a protein–lipid complex covering alveolar surfaces, which helps to prevent the collapse of alveoli [7]. Four key proteins participate in the formation of the protein–lipid complex, including surfactant protein A, B, C and D (SFTP-A, B, C, and D). These proteins are mainly yielded by alveolar epithelial type II cells, with abundance in pulmonary alveoli [8]. SFTPA and SFTPD are hydrophilic proteins that play an essential role in the regulation of innate immune systems in the lung [8,9]. For instance, SFTPD can deter the dissemination of infectious microbes by suppressing the agglutination and growth inhibition of the microbes [10], as well as through enhancing phagocytosis in macrophages [11]. Inversely, SFTPB and SFTPC are hydrophobic proteins with the capacity of mitigating surface tension in the lung [12]. Strikingly, it has been proposed that SFTPD expression was associated with tumour progression and survival of patients with lung cancer [13–15]. Furthermore, recent evidence also showed that SFTPD could suppress the progression of pancreatic cancer by inducing epithelial-mesenchymal-transition (EMT) and apoptosis of pancreatic cancer cells [16,17]. In addition, it has also been suggested that SFTPA and SFTPB might function as tumour suppressor genes and their dysregulation were closely related to poor prognosis of lung cancer patients [18–21]. As for SFTPC, one previous study reported that SFTPC deletion was observed in NSCLC tissues, implying that SFTPC downregulation might be involved in the progression of lung cancer [22]. However, it remains unclear whether SFTPC also has an inhibitory effect on lung cancer progression as the other surfactant proteins do.
Chronic interstitial lung diseases in children: diagnosis approaches
Published in Expert Review of Respiratory Medicine, 2018
Nadia Nathan, Laura Berdah, Keren Borensztajn, Annick Clement
Genetic factors are important contributors to pediatric ILD. Genetic variations have been mainly described in genes encoding (or interacting with) the surfactant proteins (SP): SP-C (SFTPC) and the ATP-binding cassette-family A-member 3 (ABCA3) (ABCA3), and less frequently in the genes encoding NKX homoeobox 2 (NKX2)-1, (NKX2-1), SP-B (SFTPB), SP-A (SFTPA). In situations of pulmonary alveolar proteinosis (PAP), genetic variations in genes encoding the methionyl-tRNA synthetase (MARS) (MARS) and the granulocyte-macrophage colony-stimulating factor (GM-CSF) receptors (CSF2RA and CSF2RB) have been reported [69].