Surfaktantni protein C (SP-C) jedan je od plućnih surfaktantnih proteina. To je membranski protein koji je kod ljudi kodiran genom SFTPC.[5][6][7]
Kratke činjenice Dostupne strukture, PDB ...
Zatvori
Dužina polipeptidnog lanca je 197 aminokiselina, а molekulska težina 21.053 Da.[8]
10 | | 20 | | 30 | | 40 | | 50 |
MDVGSKEVLM | | ESPPDYSAAP | | RGRFGIPCCP | | VHLKRLLIVV | | VVVVLIVVVI |
VGALLMGLHM | | SQKHTEMVLE | | MSIGAPEAQQ | | RLALSEHLVT | | TATFSIGSTG |
LVVYDYQQLL | | IAYKPAPGTC | | CYIMKIAPES | | IPSLEALNRK | | VHNFQMECSL |
QAKPAVPTSK | | LGQAEGRDAG | | SAPSGGDPAF | | LGMAVNTLCG | | EVPLYYI |
Kratke činjenice N-terminalni propeptidni surfaktantni protein C, Identifikatori ...
N-terminalni propeptidni surfaktantni protein C |
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![Thumb](//upload.wikimedia.org/wikipedia/commons/thumb/8/84/1spf_opm.png/200px-1spf_opm.png) Svinjski SP-C koji korespondira sa ljudskim zrelim oblikom |
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Simbol | SP_C-Propep |
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Zatvori
SFTPC 197-aminokiselinski protein sastavljen od dvije polovine: jedinstvenog N-terminalnog propeptidnog domena i C-terminalnog BRICHOS domena. Propeptidni domen dužine oko 100 aa zapravo ne sadrži samo cijepani dio, već i zreli peptid. Može se dalje razgraditi na 23-aminokiselinski spiralni transmembranski propeptid, zreli izlučeni SP-C (24-58) i linker (59-89) koji se povezuje s domenom BRICHOS.[9]
Propeptid plućnog surfaktanta C ima N-terminalni alfa-heliksni segment čija je predložena funkcija bila stabilizacija proteinske strukture, budući da se zreli peptid može nepovratno transformirati iz svoje nativne alfa-heliksne strukture u agregate beta-listova i formirati amiloidna vlakna. Također je prijavljeno da tačan unutarćelijski promet proSP-C zavisi od propeptida.[10]
Struktura BRICHOS domena je riješena. Mutacije u ovom domenu također dovode do amiloidnih vlakana sačinjenih od zrelog peptida, što ukazuje na šaperonsku aktivnost.[9]
Mutacije su povezane s disfunkcijom metabolizma surfaktanta tip 2.
Ljudi i životinje rođeni bez SP-C imaju tendenciju razvoja progresivne intersticijske bolesti pluća.
Rekombinantni SP-C koristi se u Venticuteu, vještačkom surfaktantu za pluća.
Opisan je postupak masovne proizvodnje analoga zvanog rSP-C33Le, fuzijom sa spidroinom.[11]
- 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. (juli 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.
- Simatos GA, Forward KB, Morrow MR, Keough KM (juni 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 (juli 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. (januar 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 (maj 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. (novembar 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 (maj 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 (decembar 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 (februar 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. (maj 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.