ID:FA5_HUMAN DESCRIPTION: RecName: Full=Coagulation factor V; AltName: Full=Activated protein C cofactor; AltName: Full=Proaccelerin, labile factor; Contains: RecName: Full=Coagulation factor V heavy chain; Contains: RecName: Full=Coagulation factor V light chain; Flags: Precursor; FUNCTION: Central regulator of hemostasis. It serves as a critical cofactor for the prothrombinase activity of factor Xa that results in the activation of prothrombin to thrombin. ENZYME REGULATION: Inhibited by SERPINA5. SUBUNIT: Factor Va, the activated form of factor V, is composed of a heavy chain and a light chain, non-covalently bound. The interaction between the two chains is calcium-dependent. Forms heterodimer with SERPINA5. SUBCELLULAR LOCATION: Secreted (By similarity). TISSUE SPECIFICITY: Plasma. DOMAIN: Domain B contains 35 x 9 AA tandem repeats, and 2 x 17 AA repeats. PTM: Thrombin activates factor V proteolytically to the active cofactor, factor Va (formation of a heavy chain at the N-terminus and a light chain at the C-terminus). PTM: Sulfation is required for efficient thrombin cleavage and activation and for full procoagulant activity. PTM: Activated protein C inactivates factor V and factor Va by proteolytic degradation. PTM: Phosphorylation sites are present in the extracellular medium. DISEASE: Defects in F5 are the cause of factor V deficiency (FA5D) [MIM:227400]; also known as Owren parahemophilia. It is an hemorrhagic diastesis. DISEASE: Defects in F5 are the cause of thrombophilia due to activated protein C resistance (THPH2) [MIM:188055]. THPH2 is a hemostatic disorder due to defective degradation of factor Va by activated protein C. It is characterized by a poor anticoagulant response to activated protein C resulting in tendency to thrombosis. DISEASE: Defects in F5 are a cause of susceptibility to Budd- Chiari syndrome (BDCHS) [MIM:600880]. A syndrome caused by obstruction of hepatic venous outflow involving either the hepatic veins or the terminal segment of the inferior vena cava. Obstructions are generally caused by thrombosis and lead to hepatic congestion and ischemic necrosis. Clinical manifestations observed in the majority of patients include hepatomegaly, right upper quadrant pain and abdominal ascites. Budd-Chiari syndrome is associated with a combination of disease states including primary myeloproliferative syndromes and thrombophilia due to factor V Leiden, protein C deficiency and antithrombin III deficiency. Budd-Chiari syndrome is a rare but typical complication in patients with polycythemia vera. DISEASE: Defects in F5 may be a cause of susceptibility to ischemic stroke (ISCHSTR) [MIM:601367]; also known as cerebrovascular accident or cerebral infarction. A stroke is an acute neurologic event leading to death of neural tissue of the brain and resulting in loss of motor, sensory and/or cognitive function. Ischemic strokes, resulting from vascular occlusion, is considered to be a highly complex disease consisting of a group of heterogeneous disorders with multiple genetic and environmental risk factors. DISEASE: Defects in F5 are associated with susceptibility to pregnancy loss, recurrent, type 1 (RPRGL1) [MIM:614389]. RPRGL1 is a common complication of pregnancy, resulting in spontaneous abortion before the fetus has reached viability. The term includes all miscarriages from the time of conception until 24 weeks of gestation. Recurrent pregnancy loss is defined as 3 or more consecutive spontaneous abortions. SIMILARITY: Belongs to the multicopper oxidase family. SIMILARITY: Contains 3 F5/8 type A domains. SIMILARITY: Contains 2 F5/8 type C domains. SIMILARITY: Contains 6 plastocyanin-like domains. SEQUENCE CAUTION: Sequence=ABD23003.1; Type=Erroneous gene model prediction; Sequence=CAI23065.1; Type=Erroneous gene model prediction; WEB RESOURCE: Name=Wikipedia; Note=Factor V entry; URL="http://en.wikipedia.org/wiki/Factor_V"; WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/F5"; WEB RESOURCE: Name=SeattleSNPs; URL="http://pga.gs.washington.edu/data/f5/"; WEB RESOURCE: Name=SHMPD; Note=The Singapore human mutation and polymorphism database; URL="http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=F5";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P12259
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
LP895760 - Sequence 624 from Patent EP3253886. LF205428 - JP 2014500723-A/12931: Polycomb-Associated Non-Coding RNAs. LP895181 - Sequence 45 from Patent EP3253886. AK226079 - Homo sapiens mRNA for Coagulation factor V precursor variant, clone: pf09883. M16967 - Human coagulation factor V mRNA, complete cds. M14335 - Human coagulation factor V mRNA, complete cds. BC111588 - Synthetic construct Homo sapiens clone IMAGE:40080546, MGC:133409 F5 protein (F5) mRNA, encodes complete protein. AB385005 - Synthetic construct DNA, clone: pF1KB4969, Homo sapiens F5 gene for coagulation factor V precursor, complete cds, without stop codon, in Flexi system. BC111461 - Synthetic construct Homo sapiens clone IMAGE:40080642, MGC:133430 F5 protein (F5) mRNA, encodes complete protein. BC111929 - Synthetic construct Homo sapiens clone IMAGE:40080738, MGC:133451 F5 protein (F5) mRNA, encodes complete protein. BC113357 - Synthetic construct Homo sapiens clone IMAGE:40080834, MGC:133388 coagulation factor V (proaccelerin, labile factor) (F5) mRNA, encodes complete protein. LF320394 - JP 2014500723-A/127897: Polycomb-Associated Non-Coding RNAs. AJ297255 - Homo sapiens partial mRNA for coagulation factor V jinjiang B domain, exon 13. LF320393 - JP 2014500723-A/127896: Polycomb-Associated Non-Coding RNAs. LF320392 - JP 2014500723-A/127895: Polycomb-Associated Non-Coding RNAs. LF320391 - JP 2014500723-A/127894: Polycomb-Associated Non-Coding RNAs. LF320390 - JP 2014500723-A/127893: Polycomb-Associated Non-Coding RNAs. LF320391 - JP 2014500723-A/127894: Polycomb-Associated Non-Coding RNAs. LF320389 - JP 2014500723-A/127892: Polycomb-Associated Non-Coding RNAs. LF320388 - JP 2014500723-A/127891: Polycomb-Associated Non-Coding RNAs. AK291613 - Homo sapiens cDNA FLJ77149 partial cds, highly similar to Homo sapiens coagulation factor V (proaccelerin, labile factor) (F5), mRNA. M94010 - Human coagulation factor V (F5) mRNA, partial cds. LF320387 - JP 2014500723-A/127890: Polycomb-Associated Non-Coding RNAs. AJ297254 - Homo sapiens partial mRNA for coagulation factor V jinjiang A2 domain, exon 11. AK300469 - Homo sapiens cDNA FLJ50218 complete cds, highly similar to Coagulation factor V precursor. LF205427 - JP 2014500723-A/12930: Polycomb-Associated Non-Coding RNAs. JD187042 - Sequence 168066 from Patent EP1572962. JD310550 - Sequence 291574 from Patent EP1572962. JD361515 - Sequence 342539 from Patent EP1572962. MA441005 - JP 2018138019-A/12931: Polycomb-Associated Non-Coding RNAs. MA555971 - JP 2018138019-A/127897: Polycomb-Associated Non-Coding RNAs. MA555970 - JP 2018138019-A/127896: Polycomb-Associated Non-Coding RNAs. MA555969 - JP 2018138019-A/127895: Polycomb-Associated Non-Coding RNAs. MA555968 - JP 2018138019-A/127894: Polycomb-Associated Non-Coding RNAs. MA555967 - JP 2018138019-A/127893: Polycomb-Associated Non-Coding RNAs. MA555968 - JP 2018138019-A/127894: Polycomb-Associated Non-Coding RNAs. MA555966 - JP 2018138019-A/127892: Polycomb-Associated Non-Coding RNAs. MA555965 - JP 2018138019-A/127891: Polycomb-Associated Non-Coding RNAs. MA555964 - JP 2018138019-A/127890: Polycomb-Associated Non-Coding RNAs. MA441004 - JP 2018138019-A/12930: Polycomb-Associated Non-Coding RNAs.
Biochemical and Signaling Pathways
BioCarta from NCI Cancer Genome Anatomy Project h_intrinsicPathway - Intrinsic Prothrombin Activation Pathway h_extrinsicPathway - Extrinsic Prothrombin Activation Pathway
Reactome (by CSHL, EBI, and GO)
Protein P12259 (Reactome details) participates in the following event(s):
R-HSA-140696 factor V -> factor Va + factor V activation peptide R-HSA-5591040 Activated protein C cleaves Factor Va intermediate form for Factor Va R-HSA-141026 Activated protein C cleaves factor Va to factor Vi intermediate form R-HSA-140686 factor Va + factor Xa -> Va:Xa complex (prothrombinase) R-HSA-481007 Exocytosis of platelet alpha granule contents R-HSA-5694431 Hexameric LMAN1:MCFD2 bind glycosylated Factor V and VIII precursors R-HSA-8952289 FAM20C phosphorylates FAM20C substrates R-HSA-140664 prothrombin -> activated thrombin (factor IIa) + thrombin activation peptide (prothrombinase catalyst) R-HSA-5694522 Inner coat assembly and cargo binding R-HSA-5694527 Loss of SAR1B GTPase R-HSA-5694446 BET1:GOSR2:STX5 bind v-SNARES on tethered vesicle R-HSA-5694417 SEC16 complex binds SAR1B:GTP:SEC23:SEC24 R-HSA-5694441 CSNK1D phosphorylates SEC23 R-HSA-5694439 COPII coat binds TRAPPCII and RAB1:GDP R-HSA-203973 Vesicle budding R-HSA-204008 SEC31:SEC13 and v-SNARE recruitment R-HSA-5694418 RAB1:GTP binds USO1 and GORASP1:GOLGA2 R-HSA-5694409 Nucleotide exchange on RAB1 R-HSA-140875 Common Pathway of Fibrin Clot Formation R-HSA-114608 Platelet degranulation R-HSA-140877 Formation of Fibrin Clot (Clotting Cascade) R-HSA-5694530 Cargo concentration in the ER R-HSA-76005 Response to elevated platelet cytosolic Ca2+ R-HSA-109582 Hemostasis R-HSA-199977 ER to Golgi Anterograde Transport R-HSA-381426 Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs) R-HSA-8957275 Post-translational protein phosphorylation R-HSA-76002 Platelet activation, signaling and aggregation R-HSA-204005 COPII-mediated vesicle transport R-HSA-199991 Membrane Trafficking R-HSA-948021 Transport to the Golgi and subsequent modification R-HSA-392499 Metabolism of proteins R-HSA-597592 Post-translational protein modification R-HSA-5653656 Vesicle-mediated transport R-HSA-446203 Asparagine N-linked glycosylation
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
