Komponenta komplementa 3

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Komponenta komplementa 3

PDB prikaz baziran na 1c3d.
Dostupne strukture
1c3d, 1ghq, 1w2s, 2a73, 2a74, 2b39, 2gox, 2hr0, 2i07, 2ice, 2icf
Identifikatori
SimboliC3; ASP; CPAMD1
Vanjski IDOMIM: 120700 MGI: 88227 HomoloGene: 68031 GeneCards: C3 Gene
Ontologija gena
Molekularna funkcija • aktivnost inhibitora endopeptidaze
• receptorsko vezivanje
Celularna komponenta • ekstracelularni region
Biološki proces • inflamatorni respons
• aktivacija komplementa, alternativni put
• aktivacija komplementa, klasični put
• prenos signala
• signalni put G-protein spregnutog receptora
Ortolozi
VrstaČovekMiš
Entrez71812266
EnsemblENSG00000125730ENSMUSG00000024164
UniProtP01024Q207D2
RefSeq (mRNA)NM_000064NM_009778
RefSeq (protein)NP_000055NP_033908
Lokacija (UCSC)Chr 19:
6.68 - 6.73 Mb		Chr 17:
56.89 - 56.91 Mb
PubMed pretraga[1][2]

Komponenta komplementa 3 (C3) je protein imunskog sistema. On ima centralnu ulogu u sistemu komplementa i doprinosi urođenoj imunosti. Kod ljudi je on kodiran na hromozomu 19 C3 genom.[1][2]

Funkcija

C3 ima centralnu mesto u aktivaciji sistema komplementa.[3] Njegova aktivacija je neophodna u putevima aktivacije klasičnog i alternativnnog komplementa. Ljudi sa C3 deficijencijom su podložni bakterijskim infekcijama.[4][5]

Struktura

Nekoliko C3 kristalnih struktura je rešeno.[6] Ovaj protein se sastoji od 13 domena.[7][8][9][10]

Interakcije

Za komponenta komplementa 3 je pokazano da interaguje sa faktorom H.[11][12]

Literatura

  1. ↑ de Bruijn MH, Fey GH (February 1985). „Human complement component C3: cDNA coding sequence and derived primary structure”. Proc. Natl. Acad. Sci. U.S.A. 82 (3): 708–12. DOI:10.1073/pnas.82.3.708. PMC 397115. PMID 2579379. 
  2. ↑ „Entrez Gene: C3 complement component 3”. 
  3. ↑ Sahu A, Lambris JD (April 2001). „Structure and biology of complement protein C3, a connecting link between innate and acquired immunity”. Immunol. Rev. 180: 35–48. DOI:10.1034/j.1600-065X.2001.1800103.x. PMID 11414361. 
  4. ↑ Lachmann P (December 1975). „Genetics of the complement system”. J. Med. Genet. 12 (4): 372–7. DOI:10.1136/jmg.12.4.372. PMC 1013316. PMID 768477. 
  5. ↑ Matsuyama W, Nakagawa M, Takashima H, Muranaga F, Sano Y, Osame M (December 2001). „Molecular analysis of hereditary deficiency of the third component of complement (C3) in two sisters”. Intern. Med. 40 (12): 1254–8. DOI:10.2169/internalmedicine.40.1254. PMID 11813855. 
  6. ↑ „Advanced search results for UniProt accession P01024”. Protein Data Bank in Europe (PDBe). European Bioinformatics Institute. Pristupljeno 27. 12. 2010. 
  7. ↑ Janssen BJ, Huizinga EG, Raaijmakers HC, Roos A, Daha MR, Nilsson-Ekdahl K, Nilsson B, Gros P (September 2005). „Structures of complement component C3 provide insights into the function and evolution of immunity”. Nature 437 (7058): 505–11. DOI:10.1038/nature04005. PMID 16177781. 
  8. ↑ Janssen BJ, Christodoulidou A, McCarthy A, Lambris JD, Gros P (November 2006). „Structure of C3b reveals conformational changes that underlie complement activity”. Nature 444 (7116): 213–6. DOI:10.1038/nature05172. PMID 17051160. 
  9. ↑ Wiesmann C, Katschke KJ, Yin J, Helmy KY, Steffek M, Fairbrother WJ, McCallum SA, Embuscado L, DeForge L, Hass PE, van Lookeren Campagne M (November 2006). „Structure of C3b in complex with CRIg gives insights into regulation of complement activation”. Nature 444 (7116): 217–20. DOI:10.1038/nature05263. PMID 17051150. 
  10. ↑ Fredslund F, Jenner L, Husted LB, Nyborg J, Andersen GR, Sottrup-Jensen L (August 2006). „The structure of bovine complement component 3 reveals the basis for thioester function”. J. Mol. Biol. 361 (1): 115–27. DOI:10.1016/j.jmb.2006.06.009. PMID 16831446. 
  11. ↑ Soames, C J; Sim R B (September 1997). „Interactions between human complement components factor H, factor I and C3b”. Biochem. J. (ENGLAND) 326 ( Pt 2): 553–61. ISSN 0264-6021. PMC 1218704. PMID 9291131. 
  12. ↑ Jokiranta, T S; Westin J, Nilsson U R, Nilsson B, Hellwage J, Löfås S, Gordon D L, Ekdahl K N, Meri S (March 2001). „Complement C3b interactions studied with surface plasmon resonance technique”. Int. Immunopharmacol. (Netherlands) 1 (3): 495–506. DOI:10.1016/S1567-5769(00)00042-4. ISSN 1567-5769. PMID 11367533. 

Dodatna literatura

  • Bradley, D T; Zipfel, P F; Hughes, A E (2011). „Complement in age-related macular degeneration: a focus on function”. Eye. DOI:10.1038/eye.2011.37. 

Spoljašnje veze

  • Atipični hemolitički-uremički sindrom
  • OMIM
  • MeSH Complement+C3
  • p
  • r
  • u
Aktivatori/enzimi
Rano
C: C1Q/C1R/C1S - C4 (C4a) - C2

L: MASP1/MASP2 - MBL

A: Faktor B - Faktor D - Faktor P/Properdin
Srednje
C3 (C3a, C3b/iC3b) - C5 (C5a)
C3-konvertaza - C5-konvertaza
Kasno
MAC (C6, C7, C8, C9)
Inhibitori
CLA: C1-inhibitor - Faktor ubrzanja raspada/CD59 - Faktor I

CL: C4BP

A: Faktor H
Receptori komplementa
CR1 - CR2 - CR3 - CR4 - CD11b/CD11c/CD18 - Anafilatoksin (C3a, C5a)

M: LMC

ćelije/fis/auag/auab

imdf/ipig/hyps/tumr

proc, lek (L3/4)