SFTPC is a 197-residue protein made up of two halves: a unique N-terminal propeptide domain and a C-terminal BRICHOS domain. The around 100-aa long propeptide domain actually contains not only the cleaved part, but also the mature peptide. It can be further broken down into a 23-aa helical transmembrane propeptide proper, the mature secreted SP-C (24-58), and a linker (59-89) that connects to the BRICHOS domain.[8]
The propeptide of pulmonary surfactant C has an N-terminal alpha-helical segment whose suggested function was stabilization of the protein structure, since the mature peptide 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.[9]
The structure of the BRICHOS domain has been solved. Mutations in this domain also lead to amyloid fibrils made up of the mature peptide, suggesting a chaperone activity.[8]
Recombinant SP-C is used in Venticute, an artificial lung surfactant.
A process to mass-produce an analogue called rSP-C33Le by fusion with spidroin has been described.[10]
Referencesedit
^ abcGRCh38: Ensembl release 89: ENSG00000168484 - Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000022097 - Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^Keller A, Eistetter HR, Voss T, Schäfer KP (July 1991). "The pulmonary surfactant protein C (SP-C) precursor is a type II transmembrane protein". The Biochemical Journal. 277 ( Pt 2) (Pt 2): 493–9. doi:10.1042/bj2770493. PMC1151261. PMID 1859376.
^Johansson H, Nordling K, Weaver TE, Johansson J (July 2006). "The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment". The Journal of Biological Chemistry. 281 (30): 21032–9. doi:10.1074/jbc.M603001200. PMID 16709565.
^"Entrez Gene: SFTPC surfactant, pulmonary-associated protein C".
^ abWillander H, Askarieh G, Landreh M, Westermark P, Nordling K, Keränen H, et al. (February 2012). "High-resolution structure of a BRICHOS domain and its implications for anti-amyloid chaperone activity on lung surfactant protein C". Proceedings of the National Academy of Sciences of the United States of America. 109 (7): 2325–9. Bibcode:2012PNAS..109.2325W. doi:10.1073/pnas.1114740109. PMC3289314. PMID 22308375.
^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". The FEBS Journal. 273 (5): 926–35. doi:10.1111/j.1742-4658.2006.05124.x. PMID 16478467. S2CID 1231483.
^Kronqvist N, Sarr M, Lindqvist A, Nordling K, Otikovs M, Venturi L, et al. (May 2017). "Efficient protein production inspired by how spiders make silk". Nature Communications. 8 (1): 15504. Bibcode:2017NatCo...815504K. doi:10.1038/ncomms15504. PMC5457526. PMID 28534479.
Further readingedit
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". Annual Review of Physiology. 66: 601–23. doi:10.1146/annurev.physiol.66.032102.134711. PMID 14977415.
Brasch F, Griese M, Tredano M, Johnen G, Ochs M, Rieger C, et al. (July 2004). "Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene". The European Respiratory Journal. 24 (1): 30–9. doi:10.1183/09031936.04.00000104. PMID 15293602.
Curstedt T, Johansson J, Persson P, Eklund A, Robertson B, Löwenadler B, Jörnvall H (April 1990). "Hydrophobic surfactant-associated polypeptides: SP-C is a lipopeptide with two palmitoylated cysteine residues, whereas SP-B lacks covalently linked fatty acyl groups". Proceedings of the National Academy of Sciences of the United States of America. 87 (8): 2985–9. Bibcode:1990PNAS...87.2985C. doi:10.1073/pnas.87.8.2985. PMC53818. PMID 2326260.
Simatos GA, Forward KB, Morrow MR, Keough KM (June 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, Pilot-Matias TJ, Kister SE, Whitsett JA (July 1988). "Two SP-C genes encoding human pulmonary surfactant proteolipid". The Journal of Biological Chemistry. 263 (21): 10326–31. doi:10.1016/S0021-9258(19)81519-3. PMID 2839484.
Glasser SW, Korfhagen TR, Weaver TE, Clark JC, Pilot-Matias T, Meuth J, et al. (January 1988). "cDNA, deduced polypeptide structure and chromosomal assignment of human pulmonary surfactant proteolipid, SPL(pVal)". The Journal of Biological Chemistry. 263 (1): 9–12. doi:10.1016/S0021-9258(19)57347-1. PMID 3335510.
Johansson J, Jörnvall H, Eklund A, Christensen N, Robertson B, Curstedt T (May 1988). "Hydrophobic 3.7 kDa surfactant polypeptide: structural characterization of the human and bovine forms". FEBS Letters. 232 (1): 61–4. doi:10.1016/0014-5793(88)80386-7. PMID 3366248. S2CID 29388224.
Warr RG, Hawgood S, Buckley DI, Crisp TM, Schilling J, Benson BJ, et al. (November 1987). "Low molecular weight human pulmonary surfactant protein (SP5): isolation, characterization, and cDNA and amino acid sequences". Proceedings of the National Academy of Sciences of the United States of America. 84 (22): 7915–9. Bibcode:1987PNAS...84.7915W. doi:10.1073/pnas.84.22.7915. PMC299446. PMID 3479771.
Young WA (May 1966). "Familial fibrocystic pulmonary dysplasia: a new case in a known affected family". Canadian Medical Association Journal. 94 (20): 1059–61. PMC1935417. PMID 5942662.
Wood S, Yaremko ML, Schertzer M, Kelemen PR, Minna J, Westbrook CA (December 1994). "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". Experimental Lung Research. 20 (1): 57–72. doi:10.3109/01902149409064373. PMID 8181452.
Nogee LM, Dunbar AE, Wert SE, Askin F, Hamvas A, Whitsett JA (February 2001). "A mutation in the surfactant protein C gene associated with familial interstitial lung disease". The New England Journal of Medicine. 344 (8): 573–9. doi:10.1056/NEJM200102223440805. PMID 11207353.
Glasser SW, Burhans MS, Korfhagen TR, Na CL, Sly PD, Ross GF, et al. (May 2001). "Altered stability of pulmonary surfactant in SP-C-deficient mice". Proceedings of the National Academy of Sciences of the United States of America. 98 (11): 6366–71. Bibcode:2001PNAS...98.6366G. doi:10.1073/pnas.101500298. PMC33474. PMID 11344267.
External linksedit
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)
This article incorporates text from the public domain Pfam and InterPro: IPR015091
This article on a gene on human chromosome 8 is a stub. You can help Wikipedia by expanding it.
surfactant, protein, pulmonary, surfactant, proteins, humans, this, encoded, sftpc, gene, sftpcavailable, structurespdbortholog, search, pdbe, rcsblist, codes2yadidentifiersaliasessftpc, bricd6, sftp2, smdp2, surfactant, protein, sp5external, idsomim, 178620, . Surfactant protein C SP C is one of the pulmonary surfactant proteins In humans this is encoded by the SFTPC gene 5 6 7 SFTPCAvailable structuresPDBOrtholog search PDBe RCSBList of PDB id codes2YADIdentifiersAliasesSFTPC BRICD6 PSP C SFTP2 SMDP2 SP C surfactant protein C SP5External IDsOMIM 178620 MGI 109517 HomoloGene 2271 GeneCards SFTPCGene location Human Chr Chromosome 8 human 1 Band8p21 3Start22 156 913 bp 1 End22 164 479 bp 1 Gene location Mouse Chr Chromosome 14 mouse 2 Band14 D2 14 36 32 cMStart70 758 389 bp 2 End70 761 521 bp 2 RNA expression patternBgeeHumanMouse ortholog Top expressed inlower lobe of lungright lungupper lobe of lungupper lobe of left lungvisceral pleuraparotid glandinternal globus pallidusvena cavaputamenright ventricleTop expressed inright lung lobeleft lung lobealveolar ductsexually immature organismlipatrioventricular valveadrenal glandcarotid bodymoruladigastric muscleMore reference expression dataBioGPSMore reference expression dataGene ontologyMolecular functionprotein binding identical protein bindingCellular componentextracellular region clathrin coated endocytic vesicle lamellar granule endoplasmic reticulum membrane multivesicular body lumen extracellular spaceBiological processrespiratory gaseous exchange by respiratory systemSources Amigo QuickGOOrthologsSpeciesHumanMouseEntrez644020389EnsemblENSG00000168484ENSMUSG00000022097UniProtP11686P21841Q6P8P8RefSeq mRNA NM 001172357NM 001172410NM 003018NM 001317778NM 001317779NM 001317780NM 011359RefSeq protein NP 001165828NP 001165881NP 001304707NP 001304708NP 001304709NP 003009NP 035489Location UCSC Chr 8 22 16 22 16 MbChr 14 70 76 70 76 MbPubMed search 3 4 WikidataView Edit HumanView Edit MouseIt is a membrane protein Contents 1 Structure 2 Clinical significance 3 References 4 Further reading 5 External linksStructure editSurfactant protein C N terminal propeptide nbsp Pig SP C corresponds to human mature formIdentifiersSymbolSP C PropepPfamPF08999InterProIPR001729PROSITEPDOC00298OPM superfamily69OPM protein1spfAvailable protein structures Pfam structures ECOD PDBRCSB PDB PDBe PDBjPDBsumstructure summarySFTPC is a 197 residue protein made up of two halves a unique N terminal propeptide domain and a C terminal BRICHOS domain The around 100 aa long propeptide domain actually contains not only the cleaved part but also the mature peptide It can be further broken down into a 23 aa helical transmembrane propeptide proper the mature secreted SP C 24 58 and a linker 59 89 that connects to the BRICHOS domain 8 The propeptide of pulmonary surfactant C has an N terminal alpha helical segment whose suggested function was stabilization of the protein structure since the mature peptide 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 9 The structure of the BRICHOS domain has been solved Mutations in this domain also lead to amyloid fibrils made up of the mature peptide suggesting a chaperone activity 8 Clinical significance editMutations are associated with surfactant metabolism dysfunction type 2 Humans and animals born lacking SP C tend to develop progressive interstitial lung disease Recombinant SP C is used in Venticute an artificial lung surfactant A process to mass produce an analogue called rSP C33Le by fusion with spidroin has been described 10 References edit a b c GRCh38 Ensembl release 89 ENSG00000168484 Ensembl May 2017 a b c GRCm38 Ensembl release 89 ENSMUSG00000022097 Ensembl May 2017 Human PubMed Reference National Center for Biotechnology Information U S National Library of Medicine Mouse PubMed Reference National Center for Biotechnology Information U S National Library of Medicine Keller A Eistetter HR Voss T Schafer KP July 1991 The pulmonary surfactant protein C SP C precursor is a type II transmembrane protein The Biochemical Journal 277 Pt 2 Pt 2 493 9 doi 10 1042 bj2770493 PMC 1151261 PMID 1859376 Johansson H Nordling K Weaver TE Johansson J July 2006 The Brichos domain containing C terminal part of pro surfactant protein C binds to an unfolded poly val transmembrane segment The Journal of Biological Chemistry 281 30 21032 9 doi 10 1074 jbc M603001200 PMID 16709565 Entrez Gene SFTPC surfactant pulmonary associated protein C a b Willander H Askarieh G Landreh M Westermark P Nordling K Keranen H et al February 2012 High resolution structure of a BRICHOS domain and its implications for anti amyloid chaperone activity on lung surfactant protein C Proceedings of the National Academy of Sciences of the United States of America 109 7 2325 9 Bibcode 2012PNAS 109 2325W doi 10 1073 pnas 1114740109 PMC 3289314 PMID 22308375 Li J Liepinsh E Almlen A Thyberg J Curstedt T Jornvall H Johansson J March 2006 Structure and influence on stability and activity of the N terminal propeptide part of lung surfactant protein C The FEBS Journal 273 5 926 35 doi 10 1111 j 1742 4658 2006 05124 x PMID 16478467 S2CID 1231483 Kronqvist N Sarr M Lindqvist A Nordling K Otikovs M Venturi L et al May 2017 Efficient protein production inspired by how spiders make silk Nature Communications 8 1 15504 Bibcode 2017NatCo 815504K doi 10 1038 ncomms15504 PMC 5457526 PMID 28534479 Further reading editPerez Gil J 2002 Lipid protein interactions of hydrophobic proteins SP B and SP C in lung surfactant assembly and dynamics Pediatric Pathology amp 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 amp 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 amp 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 Annual Review of Physiology 66 601 23 doi 10 1146 annurev physiol 66 032102 134711 PMID 14977415 Brasch F Griese M Tredano M Johnen G Ochs M Rieger C et al July 2004 Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene The European Respiratory Journal 24 1 30 9 doi 10 1183 09031936 04 00000104 PMID 15293602 Curstedt T Johansson J Persson P Eklund A Robertson B Lowenadler B Jornvall H April 1990 Hydrophobic surfactant associated polypeptides SP C is a lipopeptide with two palmitoylated cysteine residues whereas SP B lacks covalently linked fatty acyl groups Proceedings of the National Academy of Sciences of the United States of America 87 8 2985 9 Bibcode 1990PNAS 87 2985C doi 10 1073 pnas 87 8 2985 PMC 53818 PMID 2326260 Simatos GA Forward KB Morrow MR Keough KM June 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 Pilot Matias TJ Kister SE Whitsett JA July 1988 Two SP C genes encoding human pulmonary surfactant proteolipid The Journal of Biological Chemistry 263 21 10326 31 doi 10 1016 S0021 9258 19 81519 3 PMID 2839484 Glasser SW Korfhagen TR Weaver TE Clark JC Pilot Matias T Meuth J et al January 1988 cDNA deduced polypeptide structure and chromosomal assignment of human pulmonary surfactant proteolipid SPL pVal The Journal of Biological Chemistry 263 1 9 12 doi 10 1016 S0021 9258 19 57347 1 PMID 3335510 Johansson J Jornvall H Eklund A Christensen N Robertson B Curstedt T May 1988 Hydrophobic 3 7 kDa surfactant polypeptide structural characterization of the human and bovine forms FEBS Letters 232 1 61 4 doi 10 1016 0014 5793 88 80386 7 PMID 3366248 S2CID 29388224 Warr RG Hawgood S Buckley DI Crisp TM Schilling J Benson BJ et al November 1987 Low molecular weight human pulmonary surfactant protein SP5 isolation characterization and cDNA and amino acid sequences Proceedings of the National Academy of Sciences of the United States of America 84 22 7915 9 Bibcode 1987PNAS 84 7915W doi 10 1073 pnas 84 22 7915 PMC 299446 PMID 3479771 Young WA May 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 Kelemen PR Minna J Westbrook CA December 1994 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 Experimental Lung Research 20 1 57 72 doi 10 3109 01902149409064373 PMID 8181452 Nogee LM Dunbar AE Wert SE Askin F Hamvas A Whitsett JA February 2001 A mutation in the surfactant protein C gene associated with familial interstitial lung disease The New England Journal of Medicine 344 8 573 9 doi 10 1056 NEJM200102223440805 PMID 11207353 Glasser SW Burhans MS Korfhagen TR Na CL Sly PD Ross GF et al May 2001 Altered stability of pulmonary surfactant in SP C deficient mice Proceedings of the National Academy of Sciences of the United States of America 98 11 6366 71 Bibcode 2001PNAS 98 6366G doi 10 1073 pnas 101500298 PMC 33474 PMID 11344267 External links editGeneReviews 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 This article incorporates text from the public domain Pfam and InterPro IPR015091 nbsp This article on a gene on human chromosome 8 is a stub You can help Wikipedia by expanding it vte Retrieved from https en wikipedia org w index php title Surfactant protein C amp oldid 1170223842, 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