Surfactant protein C
| Surfactant protein C, N terminal propeptide | |||||||||
|---|---|---|---|---|---|---|---|---|---|
 Pulmonary surfactant-associated polypeptide SP-C Protein | |||||||||
| Identifiers | |||||||||
| Symbol | SP_C-Propep | ||||||||
| Pfam | PF08999 | ||||||||
| InterPro | IPR001729 | ||||||||
| PROSITE | PDOC00298 | ||||||||
| OPM superfamily | 69 | ||||||||
| OPM protein | 1spf | ||||||||
| |||||||||
| surfactant, pulmonary-associated protein C | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | SFTPC | ||||||
| Alt. symbols | SFTP2 | ||||||
| NCBI gene | 6440 | ||||||
| HGNC | 10802 | ||||||
| OMIM | 178620 | ||||||
| RefSeq | NM_003018 | ||||||
| UniProt | P11686 | ||||||
| Other data | |||||||
| Locus | Chr. 8 p21 | ||||||
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Surfactant protein C (SP-C), is one of the pulmonary surfactant proteins. In humans this is encoded by the SFTPC gene.[1][2][3]
It is a membrane protein which manufactures surfactant.
The propeptide of pulmonary surfactant C has an N-terminal alpha-helical segment whose suggested function was stabilization of the protein structure, since the latter can irreversibly transform from its native alpha-helical structure to beta-sheet aggregates and form amyloid fibrils. The correct intracellular trafficking of proSP-C has also been reported to depend on the propeptide.[4]
Clinical significance
It is associated with surfactant metabolism dysfunction type 2.
Humans and animals born lacking SP-C tend to develop progressive interstitial lung disease.
References
- ^ Keller A, Eistetter HR, Voss T, Schafer KP (Aug 1991). "The pulmonary surfactant protein C (SP-C) precursor is a type II transmembrane protein". Biochem J. 277 (Pt 2): 493–9. PMC 1151261. PMID 1859376.
- ^ Johansson H, Nordling K, Weaver TE, Johansson J (Jul 2006). "The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment". J Biol Chem. 281 (30): 21032–9. doi:10.1074/jbc.M603001200. PMID 16709565.
{{cite journal}}: CS1 maint: unflagged free DOI (link) - ^ "Entrez Gene: SFTPC surfactant, pulmonary-associated protein C".
- ^ Li J, Liepinsh E, Almlén A, Thyberg J, Curstedt T, Jörnvall H, Johansson J (March 2006). "Structure and influence on stability and activity of the N-terminal propeptide part of lung surfactant protein C". FEBS J. 273 (5): 926–35. doi:10.1111/j.1742-4658.2006.05124.x. PMID 16478467.
External links
- GeneReviews/NIH/NCBI/UW entry on Pulmonary Fibrosis, Familial
- Pulmonary+Surfactant-Associated+Protein+B at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
Further reading
PDB gallery | |
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