Summary: Surfactant protein C, N terminal propeptide
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Surfactant protein C Edit Wikipedia article
| Surfactant protein C, N terminal propeptide | |||||||||
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Pulmonary surfactant-associated polypeptide SP-C Protein
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| Identifiers | |||||||||
| Symbol | SP_C-Propep | ||||||||
| Pfam | PF08999 | ||||||||
| InterPro | IPR001729 | ||||||||
| PROSITE | PDOC00298 | ||||||||
| OPM superfamily | 75 | ||||||||
| OPM protein | 1spf | ||||||||
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| surfactant, pulmonary-associated protein C | |
|---|---|
| Identifiers | |
| Symbol | SFTPC |
| Alt. symbols | SFTP2 |
| Entrez | 6440 |
| HUGO | 10802 |
| OMIM | 178620 |
| RefSeq | NM_003018 |
| UniProt | P11686 |
| Other data | |
| Locus | Chr. 8 p21 |
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.
- ^ "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 US National Library of Medicine Medical Subject Headings (MeSH)
Further reading
- Pérez-Gil J (2002). "Lipid-protein interactions of hydrophobic proteins SP-B and SP-C in lung surfactant assembly and dynamics". Pediatric pathology & molecular medicine. 20 (6): 445–69. doi:10.1080/15227950152625783. PMID 11699574.
- Solarin KO, Wang WJ, Beers MF (2002). "Synthesis and post-translational processing of surfactant protein C". Pediatric pathology & molecular medicine. 20 (6): 471–500. doi:10.1080/15227950152625792. PMID 11699575.
- Johansson J, Curstedt T, Robertson B (2002). "Artificial surfactants based on analogues of SP-B and SP-C". Pediatric pathology & molecular medicine. 20 (6): 501–18. doi:10.1080/15227950152625800. PMID 11699576.
- Nogee LM (2004). "Alterations in SP-B and SP-C expression in neonatal lung disease". Annu. Rev. Physiol. 66: 601–23. doi:10.1146/annurev.physiol.66.032102.134711. PMID 14977415.
- Brasch F, Griese M, Tredano M, et al. (2005). "Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene". Eur. Respir. J. 24 (1): 30–9. doi:10.1183/09031936.04.00000104. PMID 15293602.
- Curstedt T, Johansson J, Persson P, et al. (1990). "Hydrophobic surfactant-associated polypeptides: SP-C is a lipopeptide with two palmitoylated cysteine residues, whereas SP-B lacks covalently linked fatty acyl groups". Proc. Natl. Acad. Sci. U.S.A. 87 (8): 2985–9. doi:10.1073/pnas.87.8.2985. PMC 53818 . PMID 2326260.
- Simatos GA, Forward KB, Morrow MR, Keough KM (1990). "Interaction between perdeuterated dimyristoylphosphatidylcholine and low molecular weight pulmonary surfactant protein SP-C". Biochemistry. 29 (24): 5807–14. doi:10.1021/bi00476a023. PMID 2383558.
- Glasser SW, Korfhagen TR, Perme CM, et al. (1988). "Two SP-C genes encoding human pulmonary surfactant proteolipid". J. Biol. Chem. 263 (21): 10326–31. PMID 2839484.
- Glasser SW, Korfhagen TR, Weaver TE, et al. (1988). "cDNA, deduced polypeptide structure and chromosomal assignment of human pulmonary surfactant proteolipid, SPL(pVal)". J. Biol. Chem. 263 (1): 9–12. PMID 3335510.
- Johansson J, Jörnvall H, Eklund A, et al. (1988). "Hydrophobic 3.7 kDa surfactant polypeptide: structural characterization of the human and bovine forms". FEBS Lett. 232 (1): 61–4. doi:10.1016/0014-5793(88)80386-7. PMID 3366248.
- Warr RG, Hawgood S, Buckley DI, et al. (1987). "Low molecular weight human pulmonary surfactant protein (SP5): isolation, characterization, and cDNA and amino acid sequences". Proc. Natl. Acad. Sci. U.S.A. 84 (22): 7915–9. doi:10.1073/pnas.84.22.7915. PMC 299446 . PMID 3479771.
- Young WA (1966). "Familial fibrocystic pulmonary dysplasia: a new case in a known affected family". Canadian Medical Association Journal. 94 (20): 1059–61. PMC 1935417 . PMID 5942662.
- Wood S, Yaremko ML, Schertzer M, et al. (1995). "Mapping of the pulmonary surfactant SP5 (SFTP2) locus to 8p21 and characterization of a microsatellite repeat marker that shows frequent loss of heterozygosity in human carcinomas". Genomics. 24 (3): 597–600. doi:10.1006/geno.1994.1673. PMID 7713515.
- Hatzis D, Deiter G, deMello DE, Floros J (1994). "Human surfactant protein-C: genetic homogeneity and expression in RDS; comparison with other species". Exp. Lung Res. 20 (1): 57–72. doi:10.3109/01902149409064373. PMID 8181452.
- Nogee LM, Dunbar AE, Wert SE, et al. (2001). "A mutation in the surfactant protein C gene associated with familial interstitial lung disease". N. Engl. J. Med. 344 (8): 573–9. doi:10.1056/NEJM200102223440805. PMID 11207353.
- Glasser SW, Burhans MS, Korfhagen TR, et al. (2001). "Altered stability of pulmonary surfactant in SP-C-deficient mice". Proc. Natl. Acad. Sci. U.S.A. 98 (11): 6366–71. doi:10.1073/pnas.101500298. PMC 33474 . PMID 11344267.
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This article incorporates text from the public domain Pfam and InterPro IPR015091
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Surfactant protein C, N terminal propeptide Provide feedback
The N-terminal propeptide of surfactant protein C adopts an alpha-helical structure, with turn and extended regions. It's main function is the stabilisation of metastable surfactant protein C (SP-C), since the latter can irreversibly transform from its native alpha-helical structure to beta-sheet aggregates and form amyloid-like fibrils. The correct intracellular trafficking of proSP-C has also been reported to depend on the propeptide [1].
Literature references
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Li J, Liepinsh E, Almlen A, Thyberg J, Curstedt T, Jornvall H, Johansson J; , FEBS J. 2006;273:926-935.: Structure and influence on stability and activity of the N-terminal propeptide part of lung surfactant protein C. PUBMED:16478467 EPMC:16478467
This tab holds annotation information from the InterPro database.
InterPro entry IPR015091
The N-terminal propeptide of surfactant protein C adopts an alpha-helical structure, with turn and extended regions. Its main function is the stabilisation of metastable surfactant protein C (SP-C), since the latter can irreversibly transform from its native alpha-helical structure to beta-sheet aggregates and form amyloid-like fibrils. The correct intracellular trafficking of proSP-C has also been reported to depend on the propeptide [PUBMED:16478467].
Domain organisation
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Representative proteomes | UniProt (197) |
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| RP15 (7) |
RP35 (27) |
RP55 (53) |
RP75 (71) |
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| PP/heatmap | 1 | ||||||||
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| Seed (2) |
Full (91) |
Representative proteomes | UniProt (197) |
NCBI (361) |
Meta (0) |
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|---|---|---|---|---|---|---|---|---|---|
| RP15 (7) |
RP35 (27) |
RP55 (53) |
RP75 (71) |
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| Gzipped | |||||||||
You can also download a FASTA format file containing the full-length sequences for all sequences in the full alignment.
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Curation and family details
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Curation
| Seed source: | pdb_2esy |
| Previous IDs: | none |
| Type: | Domain |
| Sequence Ontology: | SO:0000417 |
| Author: |
Mistry J  , Sammut SJ
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| Number in seed: | 2 |
| Number in full: | 91 |
| Average length of the domain: | 83.00 aa |
| Average identity of full alignment: | 65 % |
| Average coverage of the sequence by the domain: | 35.63 % |
HMM information
| HMM build commands: |
build method: hmmbuild -o /dev/null HMM SEED
search method: hmmsearch -Z 45638612 -E 1000 --cpu 4 HMM pfamseq
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| Model details: |
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| Model length: | 96 | ||||||||||||
| Family (HMM) version: | 10 | ||||||||||||
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Structures
For those sequences which have a structure in the Protein DataBank, we use the mapping between UniProt, PDB and Pfam coordinate systems from the PDBe group, to allow us to map Pfam domains onto UniProt sequences and three-dimensional protein structures. The table below shows the structures on which the SP_C-Propep domain has been found. There are 8 instances of this domain found in the PDB. Note that there may be multiple copies of the domain in a single PDB structure, since many structures contain multiple copies of the same protein sequence.
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