- 同
- SP-C
WordNet
- the 3rd letter of the Roman alphabet (同)c
- (music) the keynote of the scale of C major
- a general-purpose programing language closely associated with the UNIX operating system
- any of a large group of nitrogenous organic compounds that are essential constituents of living cells; consist of polymers of amino acids; essential in the diet of animals for growth and for repair of tissues; can be obtained from meat and eggs and milk and legumes; "a diet high in protein"
PrepTutorEJDIC
- carbonの化学記号
- 蛋白(たんばく)質
- cesiumの化学記号
- cadmiumの化学記号
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/11/27 11:53:42」(JST)
[Wiki en表示]
| 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 |
75 |
| OPM protein |
1spf |
| Available protein structures: |
| Pfam |
structures |
| PDB |
RCSB PDB; PDBe; PDBj |
| PDBsum |
structure summary |
|
| 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]
Contents
- 1 Clinical significance
- 2 References
- 3 External links
- 4 Further reading
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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PDB gallery
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1spf: THE NMR STRUCTURE OF THE PULMONARY SURFACTANT-ASSOCIATED POLYPEPTIDE SP-C IN AN APOLAR SOLVENT CONTAINS A VALYL-RICH ALPHA-HELIX
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Protein: cell membrane proteins (other than Cell surface receptor, enzymes, and cytoskeleton)
|
| Arrestin |
|
| Membrane-spanning 4A |
- MS4A1
- MS4A2
- MS4A3
- MS4A4A
- MS4A4E
- MS4A5
- MS4A6A
- MS4A6E
- MS4A7
- MS4A8B
- MS4A9
- MS4A10
- MS4A12
- MS4A13
- MS4A14
- MS4A15
- MS4A18
|
| Myelin |
- Myelin basic protein
- Myelin proteolipid protein
- Myelin oligodendrocyte glycoprotein
- Myelin-associated glycoprotein
- Myelin protein zero
|
| Pulmonary surfactant |
- Pulmonary surfactant-associated protein B
- Pulmonary surfactant-associated protein C
|
| Tetraspanin |
- TSPAN1
- TSPAN2
- TSPAN3
- TSPAN4
- TSPAN5
- TSPAN6
- TSPAN7
- TSPAN8
- TSPAN9
- TSPAN10
- TSPAN11
- TSPAN12
- TSPAN13
- TSPAN14
- TSPAN15
- TSPAN16
- TSPAN17
- TSPAN18
- TSPAN19
- TSPAN20
- TSPAN21
- TSPAN22
- TSPAN23
- TSPAN24
- TSPAN25
- TSPAN26
- TSPAN27
- TSPAN28
- TSPAN29
- TSPAN30
- TSPAN31
- TSPAN32
- TSPAN33
- TSPAN34
|
| Other/ungrouped |
- Calnexin
- LDL-receptor-related protein-associated protein
- Neurofibromin 2
- Presenilin
- HFE
- Phospholipid transfer proteins
- Dysferlin
- STRC
- OTOF
|
|
see also other cell membrane protein disorders
|
This article incorporates text from the public domain Pfam and InterPro IPR015091
UpToDate Contents
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English Journal
- Histone deacetylase inhibitor restores surfactant protein-C expression in alveolar-epithelial type II cells and attenuates bleomycin-induced pulmonary fibrosis in vivo.
- Ota C1,2, Yamada M3, Fujino N1, Motohashi H4, Tando Y1, Takei Y1,5, Suzuki T6, Takahashi T1,5, Kamata S1,6, Makiguchi T3, Yamaya M1, Kubo H1.
- Experimental lung research.Exp Lung Res.2015 Oct;41(8):422-434. Epub 2015 Jul 7.
- AIM: Surfactant protein-C (SP-C) of alveolar epithelial type II cells (ATII) plays a key role in maintaining alveolar integrity and repair. Mutations or decreased expression of SFTPC, the gene encoding SP-C, causes ATII injury and aberrant repair of the lung tissue to develop pulmonary fibrosis. His
- PMID 26151196
- Autophagy regulates hyperoxia-induced intracellular accumulation of surfactant protein C in alveolar type II cells.
- Zhang L1, Zhao S2, Yuan LJ3, Wu HM4, Jiang H5, Zhao SM1, Luo G5, Xue XD6.
- Molecular and cellular biochemistry.Mol Cell Biochem.2015 Oct;408(1-2):181-9. doi: 10.1007/s11010-015-2494-z. Epub 2015 Jun 30.
- Surfactant protein C (SP-C) deficiency is a risk factor for hyperoxia-induced bronchopulmonary dysplasia in newborn infants. However, the role of SP-C deficiency in the process is unclear. Here, using neonatal rat BPD model and MLE-12, mouse alveolar epithelial type II cell, we examined the changes
- PMID 26122393
- A novel surfactant protein C L55F mutation associated with interstitial lung disease alters subcellular localization of proSP-C in A549 cells.
- Liu T1, Sano K2, Ogiwara N2, Kobayashi N1.
- Pediatric research.Pediatr Res.2015 Sep 16. doi: 10.1038/pr.2015.178. [Epub ahead of print]
- BACKGROUND: Heterozygous mutations of SFTPC, the gene encoding surfactant protein C (SP-C), result in interstitial lung disease (ILD). However, characterization of mutations located in the mature domain of precursor SP-C (proSP-C) is limited. This study examined the molecular pathogenesis of such a
- PMID 26375473
★リンクテーブル★
[★]
- 英
- surfactant protein
- 関
- [[]]
[★]
[★]
[★]
セシウム, caesium, cesium
[★]
カドミウム
- 関
- cadmium
[★]