A lanac koagulacijskog faktora XIII

F13A1
Dostupne strukture PDB Pretraga ortologa: PDBe RCSB Spisak PDB ID kodova 1EVU, 1EX0, 1F13, 1FIE, 1GGT, 1GGU, 1GGY, 1QRK, 4KTY
Identifikatori
Aliasi	F13A1
Vanjski ID-jevi	OMIM: 134570 MGI: 1921395 HomoloGene: 20077 GeneCards: F13A1
Lokacija gena (čovjek) Hrom. Hromosom 6 (čovjek) Bend 6p25.1 Početak 6,144,084 bp Kraj 6,321,013 bp
Lokacija gena (miš) Hrom. Hromosom 13 (miš) Bend 13|13 A3.3 Početak 37,051,152 bp Kraj 37,234,220 bp
Obrazac RNK ekspresije Više referentnih podataka o ekspresiji
Ontologija gena Molekularna funkcija • protein-glutamine gamma-glutamyltransferase activity • acyltransferase activity • vezivanje iona metala • aktivnost sa transferazom Ćelijska komponenta • citoplazma • platelet alpha granule lumen • blood microparticle • extracellular region • collagen-containing extracellular matrix Biološki proces • GO:0051357, GO:0051358, GO:0051359 peptide cross-linking • blood coagulation, fibrin clot formation • platelet degranulation • Hemostaza • Koagulacija (krv) • cytokine-mediated signaling pathway Izvori:Amigo / QuickGO
Ortolozi
Vrste	Čovjek	Miš
Entrez	2162	74145
Ensembl	ENSG00000124491	ENSMUSG00000039109
UniProt	P00488	Q8BH61
RefSeq (mRNK)	NM_000129	NM_001166391 NM_028784
RefSeq (bjelančevina)	NP_000120	NP_001159863 NP_083060
Lokacija (UCSC)	Chr 6: 6.14 – 6.32 Mb	Chr 13: 37.05 – 37.23 Mb
PubMed pretraga
Wikipodaci
Pogledaj/uredi – čovjek Pogledaj/uredi – miš

A lanac koagulacijskog faktora XIII je protein koji je kod ljudi kodiran genom F13A1 sa hromosoma 6.

Aminokiselinska sekvenca

Dužina polipeptidnog lanca je 102 aminokiseline, a molekulska težina 11 262 — Da .

• C: Cistein
• D: Asparaginska kiselina
• E: Glutaminska kiselina
• F: Fenilalanin
• G: Glicin
• H: Histidin
• I: Izoleucin
• K: Lizin
• L: Leucin
• M: Metionin
• N: Asparagin
• P: Prolin
• Q: Glutamin
• R: Arginin
• S: Serin
• T: Treonin
• V: Valin
• W: Triptofan
• Y: Tirozin

10		20		30		40		50
XAYGVPPSAW		TGSVDILLEY		RSSENPVRYG		QCWVFAGVFN		TFLRCLGIPA
RIVTNYFSAH		DNDANLQMDI		FLEEDGNVNS		KLTKDSVWYP		SIPAAGMSPK
SA

Funkcija

Ovaj 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.

Interakcije

Pokazalo se da polipeptid faktora koagulacije XIII, A1 reaguje sa F13B.

Dopunska literatura

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