Surfactant protein C: Difference between revisions

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| Symbol = SP_C-Propep
| Symbol = SP_C-Propep
| Name = Surfactant protein C, N terminal propeptide
| Name = Surfactant protein C, N terminal propeptide
| image =
| image =1spf_opm.png
| width =
| width = 200
| caption =
| caption = Pulmonary surfactant-associated polypeptide SP-C Protein
| Pfam= PF08999
| Pfam= PF08999
| InterPro= IPR015091
| InterPro= IPR001729
| SMART=
| SMART=
| Prosite =
| Prosite = PDOC00298
| SCOP =
| SCOP =
| TCDB =
| TCDB =
| OPM family=
| OPM family= 69
| OPM protein= 1spf
| OPM protein= 1spf
| PDB=
| PDB=
{{PDB3|1spf}} :1-35
}}
}}
 
{{infobox protein
 
|Name=[[Pulmonary surfactant-associated protein C|surfactant, pulmonary-associated protein C]]
 
{{protein
|Name=surfactant, pulmonary-associated protein C
|caption=
|caption=
|image=
|image=
|width=
|width=
|HGNCid=10802
|HGNCid=10802
|Symbol=[[SFTPC]]
|Symbol=[[Pulmonary surfactant-associated protein C|SFTPC]]
|AltSymbols=SFTP2
|AltSymbols=SFTP2
|EntrezGene=6440
|EntrezGene=6440
Line 39: Line 35:
}}
}}


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-
'''Surfactant protein C''' ('''SP-C'''), is one of the [[Pulmonary surfactant#Proteins|pulmonary surfactant proteins]]. In humans this is encoded by the ''SFTPC'' [[gene]].<ref name="pmid1859376">{{cite journal |vauthors=Keller A, Eistetter HR, Voss T, Schafer KP | title = The pulmonary surfactant protein C (SP-C) precursor is a type II transmembrane protein | journal = Biochem J | volume = 277 | issue =  Pt 2| pages = 493–9 |date=Aug 1991 | pmid = 1859376 | pmc = 1151261 | doi =  }}</ref><ref name="pmid16709565">{{cite journal |vauthors=Johansson H, Nordling K, Weaver TE, Johansson J | title = The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment | journal = J Biol Chem | volume = 281 | issue = 30 | pages = 21032–9 |date=Jul 2006 | pmid = 16709565 | pmc =  | doi = 10.1074/jbc.M603001200 }}</ref><ref name="entrez">{{cite web | title = Entrez Gene: SFTPC surfactant, pulmonary-associated protein C| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=6440| accessdate = }}</ref>
sheet aggregates and form amyloid-like fibrils. The correct intracellular trafficking of proSP-C has also been reported to depend on the propeptide<ref name="PUB00035469">{{cite journal |author=Li J, Jornvall H, Johansson J, Curstedt T, Liepinsh E, Almlen A, Thyberg J |title=Structure and influence on stability and activity of the N-terminal propeptide part of lung surfactant protein C |journal=FEBS J |volume=273 |issue=5 |pages=- |year=2006 |pmid=16478467}}</ref>.
 
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.<ref name="pmid16478467">{{cite journal |vauthors=Li J, Liepinsh E, Almlén A, Thyberg J, Curstedt T, Jörnvall H, Johansson J | title = Structure and influence on stability and activity of the N-terminal propeptide part of lung surfactant protein C | journal = FEBS J. | volume = 273 | issue = 5 | pages = 926–35 |date=March 2006 | pmid = 16478467 | doi = 10.1111/j.1742-4658.2006.05124.x | url =  }}</ref>
 
==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]].
 
<!-- The PBB_Summary template is automatically maintained by Protein Box Bot.  See Template:PBB_Controls to Stop updates. -->
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}}


==References==
==References==
{{reflist|2}}
{{reflist}}


==See also==
==External links==
* [[pulmonary surfactant]]
* [https://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=pf  GeneReviews/NIH/NCBI/UW entry on Pulmonary Fibrosis, Familial]
* {{MeshName|Pulmonary+Surfactant-Associated+Protein+B}}


==External links==
==Further reading==
* {{MeshName|Pulmonary+Surfactant-Associated+Protein+C}}
{{refbegin | 2}}
{{PBB_Further_reading
| citations =
*{{cite journal  | author=Pérez-Gil J |title=Lipid-protein interactions of hydrophobic proteins SP-B and SP-C in lung surfactant assembly and dynamics |journal=Pediatric pathology & molecular medicine |volume=20 |issue= 6 |pages= 445–69 |year= 2002 |pmid= 11699574 |doi=  10.1080/15227950152625783}}
*{{cite journal  |vauthors=Solarin KO, Wang WJ, Beers MF |title=Synthesis and post-translational processing of surfactant protein C |journal=Pediatric pathology & molecular medicine |volume=20 |issue= 6 |pages= 471–500 |year= 2002 |pmid= 11699575 |doi=  10.1080/15227950152625792}}
*{{cite journal  |vauthors=Johansson J, Curstedt T, Robertson B |title=Artificial surfactants based on analogues of SP-B and SP-C |journal=Pediatric pathology & molecular medicine |volume=20 |issue= 6 |pages= 501–18 |year= 2002 |pmid= 11699576 |doi=  10.1080/15227950152625800}}
*{{cite journal  | author=Nogee LM |title=Alterations in SP-B and SP-C expression in neonatal lung disease |journal=Annu. Rev. Physiol. |volume=66 |issue=  |pages= 601–23 |year= 2004 |pmid= 14977415 |doi= 10.1146/annurev.physiol.66.032102.134711 }}
*{{cite journal  | author=Brasch F |title=Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene |journal=Eur. Respir. J. |volume=24 |issue= 1 |pages= 30–9 |year= 2005 |pmid= 15293602 |doi=10.1183/09031936.04.00000104  |name-list-format=vanc| author2=Griese M  | author3=Tredano M  | display-authors=3  | last4=Johnen  | first4=G.  | last5=Ochs  | first5=M.  | last6=Rieger  | first6=C.  | last7=Mulugeta  | first7=S.  | last8=Muller  | first8=K.M.  | last9=Bahuau  | first9=M.  }}
*{{cite journal  | author=Curstedt T |title=Hydrophobic surfactant-associated polypeptides: SP-C is a lipopeptide with two palmitoylated cysteine residues, whereas SP-B lacks covalently linked fatty acyl groups |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=87 |issue= 8 |pages= 2985–9 |year= 1990 |pmid= 2326260 |doi=10.1073/pnas.87.8.2985  | pmc=53818  |name-list-format=vanc| author2=Johansson J  | author3=Persson P  | display-authors=3  | last4=Eklund  | first4=A  | last5=Robertson  | first5=B  | last6=Löwenadler  | first6=B  | last7=Jörnvall  | first7=H  }}
*{{cite journal  |vauthors=Simatos GA, Forward KB, Morrow MR, Keough KM |title=Interaction between perdeuterated dimyristoylphosphatidylcholine and low molecular weight pulmonary surfactant protein SP-C |journal=Biochemistry |volume=29 |issue= 24 |pages= 5807–14 |year= 1990 |pmid= 2383558 |doi=10.1021/bi00476a023  }}
*{{cite journal  | author=Glasser SW |title=Two SP-C genes encoding human pulmonary surfactant proteolipid |journal=J. Biol. Chem. |volume=263 |issue= 21 |pages= 10326–31 |year= 1988 |pmid= 2839484 |doi=  |name-list-format=vanc| author2=Korfhagen TR  | author3=Perme CM  | display-authors=3  | last4=Pilot-Matias  | first4=TJ  | last5=Kister  | first5=SE  | last6=Whitsett  | first6=JA  }}
*{{cite journal  | author=Glasser SW |title=cDNA, deduced polypeptide structure and chromosomal assignment of human pulmonary surfactant proteolipid, SPL(pVal) |journal=J. Biol. Chem. |volume=263 |issue= 1 |pages= 9–12 |year= 1988 |pmid= 3335510 |doi=  |name-list-format=vanc| author2=Korfhagen TR  | author3=Weaver TE  | display-authors=3  | last4=Clark  | first4=JC  | last5=Pilot-Matias  | first5=T  | last6=Meuth  | first6=J  | last7=Fox  | first7=JL  | last8=Whitsett  | first8=JA  }}
*{{cite journal  | author=Johansson J |title=Hydrophobic 3.7 kDa surfactant polypeptide: structural characterization of the human and bovine forms |journal=FEBS Lett. |volume=232 |issue= 1 |pages= 61–4 |year= 1988 |pmid= 3366248 |doi=10.1016/0014-5793(88)80386-7  |name-list-format=vanc| author2=Jörnvall H  | author3=Eklund A  | display-authors=3  | last4=Christensen  | first4=N  | last5=Robertson  | first5=B  | last6=Curstedt  | first6=T  }}
*{{cite journal  | author=Warr RG |title=Low molecular weight human pulmonary surfactant protein (SP5): isolation, characterization, and cDNA and amino acid sequences |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=84 |issue= 22 |pages= 7915–9 |year= 1987 |pmid= 3479771 |doi=10.1073/pnas.84.22.7915  | pmc=299446  |name-list-format=vanc| author2=Hawgood S  | author3=Buckley DI  | display-authors=3  | last4=Crisp  | first4=TM  | last5=Schilling  | first5=J  | last6=Benson  | first6=BJ  | last7=Ballard  | first7=PL  | last8=Clements  | first8=JA  | last9=White  | first9=RT  }}
*{{cite journal  | author=Young WA |title=Familial fibrocystic pulmonary dysplasia: a new case in a known affected family |journal=Canadian Medical Association Journal |volume=94 |issue= 20 |pages= 1059–61 |year= 1966 |pmid= 5942662 |doi=  | pmc=1935417  }}
*{{cite journal  | author=Wood S |title=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 |journal=Genomics |volume=24 |issue= 3 |pages= 597–600 |year= 1995 |pmid= 7713515 |doi= 10.1006/geno.1994.1673  |name-list-format=vanc| author2=Yaremko ML  | author3=Schertzer M  | display-authors=3  | last4=Kelemen  | first4=Pond R.  | last5=Minna  | first5=John  | last6=Westbrook  | first6=Carol A. }}
*{{cite journal  |vauthors=Hatzis D, Deiter G, deMello DE, Floros J |title=Human surfactant protein-C: genetic homogeneity and expression in RDS; comparison with other species |journal=Exp. Lung Res. |volume=20 |issue= 1 |pages= 57–72 |year= 1994 |pmid= 8181452 |doi=10.3109/01902149409064373  }}
*{{cite journal  | author=Nogee LM |title=A mutation in the surfactant protein C gene associated with familial interstitial lung disease |journal=N. Engl. J. Med. |volume=344 |issue= 8 |pages= 573–9 |year= 2001 |pmid= 11207353 |doi=10.1056/NEJM200102223440805  |name-list-format=vanc| author2=Dunbar AE  | author3=Wert SE  | display-authors=3  | last4=Askin  | first4=Frederic  | last5=Hamvas  | first5=Aaron  | last6=Whitsett  | first6=Jeffrey A.  }}
*{{cite journal  | author=Glasser SW |title=Altered stability of pulmonary surfactant in SP-C-deficient mice |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=98 |issue= 11 |pages= 6366–71 |year= 2001 |pmid= 11344267 |doi= 10.1073/pnas.101500298  | pmc=33474  |name-list-format=vanc| author2=Burhans MS  | author3=Korfhagen TR  | display-authors=3  | last4=Na  | first4=CL  | last5=Sly  | first5=PD  | last6=Ross  | first6=GF  | last7=Ikegami  | first7=M  | last8=Whitsett  | first8=JA }}
}}
{{refend}}
{{PDB Gallery|geneid=6440}}
 
{{Membrane proteins}}
 
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{{InterPro content|IPR015091}}
{{InterPro content|IPR015091}}


{{biochem-stub}}
[[Category:Protein domains]]
 


[[Category:Protein domains]]
{{gene-8-stub}}

Latest revision as of 08:34, 10 January 2019

Surfactant protein C, N terminal propeptide
File:1spf opm.png
Pulmonary surfactant-associated polypeptide SP-C Protein
Identifiers
SymbolSP_C-Propep
PfamPF08999
InterProIPR001729
PROSITEPDOC00298
OPM superfamily69
OPM protein1spf
surfactant, pulmonary-associated protein C
Identifiers
SymbolSFTPC
Alt. symbolsSFTP2
Entrez6440
HUGO10802
OMIM178620
RefSeqNM_003018
UniProtP11686
Other data
LocusChr. 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

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ "Entrez Gene: SFTPC surfactant, pulmonary-associated protein C".
  4. ↑ 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

Further reading


This article incorporates text from the public domain Pfam and InterPro: IPR015091


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