A lanac koagulacijskog faktora XIII
(Preusmjereno sa F13A1)
A lanac koagulacijskog faktora XIII je protein koji je kod ljudi kodiran genom F13A1 sa hromosoma 6.
Aminokiselinska sekvenca
urediDužina polipeptidnog lanca je 102 aminokiseline, a molekulska težina 11 262 — Da .[5]
| 10 | 20 | 30 | 40 | 50 | ||||
|---|---|---|---|---|---|---|---|---|
| XAYGVPPSAW | TGSVDILLEY | RSSENPVRYG | QCWVFAGVFN | TFLRCLGIPA | ||||
| RIVTNYFSAH | DNDANLQMDI | FLEEDGNVNS | KLTKDSVWYP | SIPAAGMSPK | ||||
| SA |
Funkcija
urediOvaj gen kodira podjedinicu A faktor koagulacije XIII . Koagulacijski faktor XIII je posljednji zimogen koji se aktivira u kaskadi koagulacije krvi. Faktor plazme XIII je heterotetramer sastavljen od po dvije A i 2 B podjedinice. A podjedinice imaju katalitsku funkciju, a B podjedinice nemaju enzimsku aktivnost i mogu služiti kao molekulski nosači plazme. Faktor trombocita XIII sastoji se od samo dvije A podjedinice, koje su identične onima iz plazme. Nakon cijepanja aktivacionog peptida trombinom i u prisustvu iona kalcija, faktor u plazmi XIII razdvoji svoje B podjedinice i daje isti aktivni enzim, faktor XIIIa, kao trombocitni faktor XIII. Ovaj enzim djeluje kao transglutaminaza da katalizuje stvaranje umreženog gama-glutamil-epsilon-lizina između molekula fibrina, čime se stabilizira fibrinski ugrušak. Također povezuje inhibitor alfa-2-plazmina, ili fibronektin, sa alfa lancima fibrina. Nedostatak faktora XIII je klasifikovan u dvije kategorije: nedostatak tipa I, karakteriziran nedostatkom i A i B podjedinica i nedostatak tipa II, karakteriziran nedostatkom same A podjedinice. Ovi defekti mogu dovesti do doživotne sklonosti krvarenju, neispravnog zacjeljivanja rana i uobičajenog pobačaja.[6]
Interakcije
urediPokazalo se da polipeptid faktora koagulacije XIII, A1 reaguje sa F13B.[7][8]
Reference
uredi- ^ a b c GRCh38: Ensembl release 89: ENSG00000124491 - Ensembl, maj 2017
- ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000039109 - Ensembl, maj 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.
- ^ "UniProt, H0Y4W5" (jezik: engleski). Arhivirano s originala, 30 -09- 2021. Pristupljeno 20-11- 2017. Provjerite vrijednost datuma u parametru:
|access-date=i|archive-date=(pomoć) - ^ "Entrez Gene: F13A1 coagulation factor XIII, A1 polypeptide".
- ^ Carrell NA, Erickson HP, McDonagh J (Jan 1989). "Electron microscopy and hydrodynamic properties of factor XIII subunits". J. Biol. Chem. 264 (1): 551–6. doi:10.1016/S0021-9258(17)31294-2. PMID 2491853.
- ^ Achyuthan KE, Rowland TC, Birckbichler PJ, Lee KN, Bishop PD, Achyuthan AM (Sep 1996). "Hierarchies in the binding of human factor XIII, factor XIIIa, and endothelial cell transglutaminase to human plasma fibrinogen, fibrin, and fibronectin". Mol. Cell. Biochem. 162 (1): 43–9. doi:10.1007/bf00250994. PMID 8905624. S2CID 23583301.
Dopunska literatura
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- Carrell NA, Erickson HP, McDonagh J (1989). "Electron microscopy and hydrodynamic properties of factor XIII subunits". J. Biol. Chem. 264 (1): 551–6. doi:10.1016/S0021-9258(17)31294-2. PMID 2491853.
- Takahashi N, Takahashi Y, Putnam FW (1986). "Primary structure of blood coagulation factor XIIIa (fibrinoligase, transglutaminase) from human placenta". Proc. Natl. Acad. Sci. U.S.A. 83 (21): 8019–23. Bibcode:1986PNAS...83.8019T. doi:10.1073/pnas.83.21.8019. PMC 386858. PMID 2877456.
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- Suzuki K, Iwata M, Ito S, Matsui K, Uchida A, Mizoi Y (1994). "Molecular basis for subtypic differences of the "a" subunit of coagulation factor XIII with description of the genesis of the subtypes". Hum. Genet. 94 (2): 129–35. doi:10.1007/bf00202857. PMID 7913909. S2CID 20800219.