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Gene Review

Sftpc  -  surfactant associated protein C

Mus musculus

Synonyms: Bricd6, Pulmonary surfactant-associated protein C, Pulmonary surfactant-associated proteolipid SPL(Val), SP-C, SP5, ...
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Disease relevance of Sftpc


High impact information on Sftpc

  • The A/Japan/57 influenza hemagglutinin (HA) was expressed in BALB/c mice under the transcriptional control of the surfactant protein C (SP-C) promoter, resulting in expression of HA in type II alveolar epithelial cells, as well as low level variable expression in other tissues, including the thymus in some of the founder lines [4].
  • Pulmonary pathology in the SP-C TNF-alpha mice bears a striking resemblance to human idiopathic pulmonary fibrosis, in which increased expression of TNF-alpha in type II epithelial cells has also been noted [5].
  • Surfactant protein C (SP-C; Sftpc) gene expression is restricted to pulmonary type II epithelial cells [1].
  • Overexpression of NmycEGFP in the epithelium under the control of surfactant protein C (Sftpc) regulatory elements expands the domain of S phase cells and upregulates numerous genes associated with growth and metabolism, as shown by transcriptional microarray [6].
  • TTF1 expression is unaltered in chimeric lungs whereas SPC and CC10 expression are attenuated in abnormally branched areas of chimeric lungs [7].

Chemical compound and disease context of Sftpc


Biological context of Sftpc


Anatomical context of Sftpc


Associations of Sftpc with chemical compounds

  • Induction of NFIen in a subset of type II cells inhibited SP-C gene expression without affecting expression of TTF-1 in doxycycline-treated double-transgenic mice [1].
  • We found recently that the hydrophobic lung surfactant protein SP-C specifically binds to the lipid A region of lipopolysaccharide (LPS) [16].
  • The effects of HIMF on SP-B and SP-C transcription activity, and on mRNA degradation, were investigated in mouse lung epithelial (MLE)-12 and C10 cells using the promoter-luciferase reporter assay and actinomycin D incubation [17].
  • We found that among the different hydrophobic components of mouse surfactant separated by gel filtration or reverse-phase HPLC, only SP-C exhibited the capacity to bind to a tritium-labeled LPS [18].
  • The LY294002 and wortmannin treatments showed decreased expression of SPC [19].

Regulatory relationships of Sftpc


Other interactions of Sftpc


Analytical, diagnostic and therapeutic context of Sftpc

  • The temporal-spatial expression of HIMF, SP-B, and SP-C in developing mouse lungs was examined by immunohistochemical staining, Western blot, and RT-PCR [17].
  • Data presented here demonstrate that such fetal lung morphogenetic cues induce MESC derivatives to incorporate into the reforming pseudoglandular-like tubular ducts, display pan-keratin and surfactant protein C (Sftpc) immunoreactivity, and express Sftpc transcripts while displaying a normal diploid karyotype in coculture [27].
  • The SP-C minimal promoter was found to contain two potential Ets binding sites, and electrophoretic mobility shift assays showed that two 20-bp wild-type oligonucleotides containing the 5'-GGA(A/T)-3' Ets consensus binding motif were shifted by nuclear extracts from MLE15 cells [28].
  • Immunocytochemistry showed that with T(3) treatment, Nkx2.1 and surfactant protein SP-C proteins became progressively localized to cuboidal epithelial cells and mesenchymal expression of Hoxb5 was reduced, a pattern resembling late fetal lung development [29].
  • Decreased SP-B was associated with low alveolar content of phosphatidylglycerol, accumulation of misprocessed SP-C proprotein in the air spaces, increased protein content in bronchoalveolar lavage fluid, and altered surfactant activity in vitro [30].


  1. Nuclear factor I/thyroid transcription factor 1 interactions modulate surfactant protein C transcription. Bachurski, C.J., Yang, G.H., Currier, T.A., Gronostajski, R.M., Hong, D. Mol. Cell. Biol. (2003) [Pubmed]
  2. Pneumonitis and emphysema in sp-C gene targeted mice. Glasser, S.W., Detmer, E.A., Ikegami, M., Na, C.L., Stahlman, M.T., Whitsett, J.A. J. Biol. Chem. (2003) [Pubmed]
  3. Increased and prolonged pulmonary fibrosis in surfactant protein C-deficient mice following intratracheal bleomycin. Lawson, W.E., Polosukhin, V.V., Stathopoulos, G.T., Zoia, O., Han, W., Lane, K.B., Li, B., Donnelly, E.F., Holburn, G.E., Lewis, K.G., Collins, R.D., Hull, W.M., Glasser, S.W., Whitsett, J.A., Blackwell, T.S. Am. J. Pathol. (2005) [Pubmed]
  4. A lung-specific neo-antigen elicits specific CD8+ T cell tolerance with preserved CD4+ T cell reactivity. Implications for immune-mediated lung disease. Enelow, R.I., Stoler, M.H., Srikiatkhachorn, A., Kerlakian, C., Agersborg, S., Whitsett, J.A., Braciale, T.J. J. Clin. Invest. (1996) [Pubmed]
  5. Expression of a tumor necrosis factor-alpha transgene in murine lung causes lymphocytic and fibrosing alveolitis. A mouse model of progressive pulmonary fibrosis. Miyazaki, Y., Araki, K., Vesin, C., Garcia, I., Kapanci, Y., Whitsett, J.A., Piguet, P.F., Vassalli, P. J. Clin. Invest. (1995) [Pubmed]
  6. Nmyc plays an essential role during lung development as a dosage-sensitive regulator of progenitor cell proliferation and differentiation. Okubo, T., Knoepfler, P.S., Eisenman, R.N., Hogan, B.L. Development (2005) [Pubmed]
  7. The transcription factor GATA6 is essential for branching morphogenesis and epithelial cell differentiation during fetal pulmonary development. Keijzer, R., van Tuyl, M., Meijers, C., Post, M., Tibboel, D., Grosveld, F., Koutsourakis, M. Development (2001) [Pubmed]
  8. Cellular antiendotoxin activities of lung surfactant protein C in lipid vesicles. Augusto, L.A., Synguelakis, M., Espinassous, Q., Lepoivre, M., Johansson, J., Chaby, R. Am. J. Respir. Crit. Care Med. (2003) [Pubmed]
  9. Five cysteine-containing compounds delay diabetic deterioration in Balb/cA mice. Hsu, C.C., Yen, H.F., Yin, M.C., Tsai, C.M., Hsieh, C.H. J. Nutr. (2004) [Pubmed]
  10. HNF-3/forkhead homologue-4 influences lung morphogenesis and respiratory epithelial cell differentiation in vivo. Tichelaar, J.W., Lim, L., Costa, R.H., Whitsett, J.A. Dev. Biol. (1999) [Pubmed]
  11. Structure and expression of the pulmonary surfactant protein SP-C gene in the mouse. Glasser, S.W., Korfhagen, T.R., Bruno, M.D., Dey, C., Whitsett, J.A. J. Biol. Chem. (1990) [Pubmed]
  12. Tumor necrosis factor-alpha inhibits surfactant protein C gene transcription. Bachurski, C.J., Pryhuber, G.S., Glasser, S.W., Kelly, S.E., Whitsett, J.A. J. Biol. Chem. (1995) [Pubmed]
  13. The bone morphogenic protein antagonist gremlin regulates proximal-distal patterning of the lung. Lu, M.M., Yang, H., Zhang, L., Shu, W., Blair, D.G., Morrisey, E.E. Dev. Dyn. (2001) [Pubmed]
  14. Transcription of the lung-specific surfactant protein C gene is mediated by thyroid transcription factor 1. Kelly, S.E., Bachurski, C.J., Burhans, M.S., Glasser, S.W. J. Biol. Chem. (1996) [Pubmed]
  15. Transcriptional elements from the human SP-C gene direct expression in the primordial respiratory epithelium of transgenic mice. Wert, S.E., Glasser, S.W., Korfhagen, T.R., Whitsett, J.A. Dev. Biol. (1993) [Pubmed]
  16. Interaction of pulmonary surfactant protein C with CD14 and lipopolysaccharide. Augusto, L.A., Synguelakis, M., Johansson, J., Pedron, T., Girard, R., Chaby, R. Infect. Immun. (2003) [Pubmed]
  17. Hypoxia-induced mitogenic factor modulates surfactant protein B and C expression in mouse lung. Tong, Q., Zheng, L., Dodd-o, J., Langer, J., Wang, D., Li, D. Am. J. Respir. Cell Mol. Biol. (2006) [Pubmed]
  18. Interaction of bacterial lipopolysaccharide with mouse surfactant protein C inserted into lipid vesicles. Augusto, L., Le Blay, K., Auger, G., Blanot, D., Chaby, R. Am. J. Physiol. Lung Cell Mol. Physiol. (2001) [Pubmed]
  19. PI3K-AKT pathway mediates growth and survival signals during development of fetal mouse lung. Wang, J., Ito, T., Udaka, N., Okudela, K., Yazawa, T., Kitamura, H. Tissue & cell. (2005) [Pubmed]
  20. Sox17 influences the differentiation of respiratory epithelial cells. Park, K.S., Wells, J.M., Zorn, A.M., Wert, S.E., Whitsett, J.A. Dev. Biol. (2006) [Pubmed]
  21. BMP4 modulates fibroblast growth factor-mediated induction of proximal and distal lung differentiation in mouse embryonic tracheal epithelium in mesenchyme-free culture. Hyatt, B.A., Shangguan, X., Shannon, J.M. Dev. Dyn. (2002) [Pubmed]
  22. Branching and differentiation defects in pulmonary epithelium with elevated Gata6 expression. Koutsourakis, M., Keijzer, R., Visser, P., Post, M., Tibboel, D., Grosveld, F. Mech. Dev. (2001) [Pubmed]
  23. Deficiency of SP-B reveals protective role of SP-C during oxygen lung injury. Ikegami, M., Weaver, T.E., Conkright, J.J., Sly, P.D., Ross, G.F., Whitsett, J.A., Glasser, S.W. J. Appl. Physiol. (2002) [Pubmed]
  24. Evidence from normal expression and targeted misexpression that bone morphogenetic protein (Bmp-4) plays a role in mouse embryonic lung morphogenesis. Bellusci, S., Henderson, R., Winnier, G., Oikawa, T., Hogan, B.L. Development (1996) [Pubmed]
  25. Surfactant lipid synthesis and lamellar body formation in glycogen-laden type II cells. Ridsdale, R., Post, M. Am. J. Physiol. Lung Cell Mol. Physiol. (2004) [Pubmed]
  26. FGF-10 induces SP-C and Bmp4 and regulates proximal-distal patterning in embryonic tracheal epithelium. Hyatt, B.A., Shangguan, X., Shannon, J.M. Am. J. Physiol. Lung Cell Mol. Physiol. (2004) [Pubmed]
  27. Embryonic stem cells form glandular structures and express surfactant protein C following culture with dissociated fetal respiratory tissue. Denham, M., Cole, T.J., Mollard, R. Am. J. Physiol. Lung Cell Mol. Physiol. (2006) [Pubmed]
  28. Erm/thyroid transcription factor 1 interactions modulate surfactant protein C transcription. Lin, S., Perl, A.K., Shannon, J.M. J. Biol. Chem. (2006) [Pubmed]
  29. Thyroid hormone affects embryonic mouse lung branching morphogenesis and cellular differentiation. Archavachotikul, K., Ciccone, T.J., Chinoy, M.R., Nielsen, H.C., Volpe, M.V. Am. J. Physiol. Lung Cell Mol. Physiol. (2002) [Pubmed]
  30. SP-B deficiency causes respiratory failure in adult mice. Melton, K.R., Nesslein, L.L., Ikegami, M., Tichelaar, J.W., Clark, J.C., Whitsett, J.A., Weaver, T.E. Am. J. Physiol. Lung Cell Mol. Physiol. (2003) [Pubmed]
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