Affinage

F5

Coagulation factor V · UniProt P12259

Length
2224 aa
Mass
251.7 kDa
Annotated
2026-06-09
100 papers in source corpus 15 papers cited in narrative 15 extracted findings
Cross-family judge vs UniProt: tie faithfulness: 6/6 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

Coagulation Factor V (F5) encodes a circulating procofactor whose activated form, FVa, is essential for thrombin generation, with its activity tightly regulated by activated protein C (APC) and protein S; loss of this regulatory cleavage is the central mechanism of inherited thrombophilia (PMID:8624369). FVa is inactivated by APC through cleavage at Arg506 and Arg306, with protein S stimulating these reactions, and intact FV additionally serves as an APC cofactor together with protein S in the inactivation of FVIIIa within the Xase complex (PMID:8624369, PMID:12091344). Pathogenic missense substitutions impair this regulation in distinct ways: FV Leiden (Arg506Gln) is activated normally but resists APC inactivation, producing lifelong hypercoagulability (PMID:8624369, PMID:12881304), FV Bonn (Ala512Val) slows Arg506 cleavage with protein S largely restoring inactivation (PMID:27090446), and variants disrupting Arg306 cleavage (FV Cambridge, FV Hong Kong) become near-completely inactivated only when protein S stimulates the alternative Arg679 cleavage (PMID:12091344). Other mutations cause quantitative FV deficiency through impaired secretion, heterodimer instability, or aberrant splicing—including FV New Brunswick (Ala221Val), which destabilizes the activated FVa heterodimer (PMID:12714495), FV Besançon (Ala2086Asp), which favors a closed C2-domain conformation that impairs phospholipid binding and secretion yet paradoxically produces a hypercoagulable, APC-resistant state (PMID:33605529), and synonymous, exonic, and deep-intronic variants that activate cryptic splice sites and are correctable by antisense morpholino or U7 snRNA approaches (PMID:25470420, PMID:24085767). FV and FVIII are exported from the ER via the calcium-dependent LMAN1–MCFD2 cargo receptor complex, in which MCFD2 is the primary cargo-binding subunit and LMAN1 acts as a shuttling carrier (PMID:19183188, PMID:36490287, PMID:21795745). A naturally occurring B-domain-truncated splice isoform, FV-Short, forms a high-affinity trimolecular complex with TFPIα and protein S that synergistically inhibits FXa; cis-acting regulatory sequences in F5 exon 13 control its production, and gain-of-function splicing variants that elevate FV-Short/TFPIα raise the anticoagulant threshold and cause bleeding (East Texas disorder) (PMID:36746318, PMID:25634741, PMID:33773040).

Mechanistic history

Synthesis pass · year-by-year structured walk · 14 steps
  1. 1996 High

    Established the central thrombophilia mechanism: FV Leiden activates normally but resists APC inactivation, explaining how a single F5 substitution drives lifelong hypercoagulability and defining FV's dual role as both procofactor and APC cofactor.

    Evidence Biochemical characterization of purified mutant FV and APC resistance testing in patient and reconstituted plasma

    PMID:8624369

    Open questions at the time
    • Does not resolve the structural basis of impaired Arg506 cleavage
    • Relative contribution of FVa-procoagulant resistance versus loss of APC cofactor function not quantified
  2. 2000 Medium

    Linked the R2 F5 haplotype to differential glycosylation, indicating genetically determined shifts in the FV1/FV2 isoform ratio modulate thrombogenic potential.

    Evidence Sequencing plus chromatographic separation and quantitation of FV isoforms in homozygous R2 carriers versus controls

    PMID:10744138

    Open questions at the time
    • No mutagenesis reconstitution to prove causality of A6755G/Ala2194Gly
    • Mechanism by which glycosylation alters function not defined
  3. 2002 High

    Defined how Arg306 cleavage and protein S cooperate, showing variants lacking Arg306 cleavage are still nearly fully inactivated when protein S stimulates the alternative Arg679 site.

    Evidence Recombinant FV Cambridge/Hong Kong mutants in purified APC inactivation, prothrombinase, and FVIIIa degradation assays with/without protein S

    PMID:12091344

    Open questions at the time
    • In vivo thrombotic risk of these variants not established
    • Structural basis of protein S stimulation of Arg679 cleavage unresolved
  4. 2003 High

    Demonstrated that FV deficiency can arise from post-secretory instability rather than synthesis or function defects, mechanistically separating quantitative deficiency from activity loss.

    Evidence Recombinant FV New Brunswick (Ala221Val) with pulse-chase, ELISA, prothrombinase, and thermal stability assays

    PMID:12714495

    Open questions at the time
    • Structural cause of accelerated chain dissociation not defined
    • In vivo turnover kinetics not measured
  5. 2003 High

    Showed FV Leiden's prothrombotic effect persists even under severe upstream coagulation factor deficiency, reinforcing APC resistance as the operative mechanism in defined plasma systems.

    Evidence Thrombin generation and FXa/FVa measurements in FV-FVII doubly deficient plasma reconstituted with purified FV variants

    PMID:12881304

    Open questions at the time
    • Clinical bleeding/thrombosis balance in combined deficiency not addressed
    • Single-lab plasma reconstitution system
  6. 2009 High

    Identified the LMAN1-MCFD2 complex as the ER-to-Golgi cargo receptor for FV and FVIII, explaining combined FV+FVIII deficiency through a trafficking rather than synthetic defect.

    Evidence Genetic identification of LMAN1/MCFD2 mutations in patients, co-immunoprecipitation, and cell trafficking assays

    PMID:19183188

    Open questions at the time
    • Precise FV binding determinants on the complex not mapped
    • Relative roles of LMAN1 versus MCFD2 not yet dissected
  7. 2011 High

    Confirmed in vivo that LMAN1 is required for efficient FV and FVIII secretion, with a partial (~50%) deficiency phenotype and ER stress, while sparing COPII vesicle formation.

    Evidence Lman1 knockout mice with plasma/platelet FV and FVIII quantification, liver ultrastructure, and in vitro COPII budding assay

    PMID:21795745

    Open questions at the time
    • Reason for residual secretion despite LMAN1 loss not explained
    • Tissue-specific contributions to plasma FV not resolved
  8. 2013 High

    Established that deep-intronic F5 splicing mutations cause severe deficiency and are correctable, opening antisense and U7 snRNA strategies validated in patient-derived cells.

    Evidence F5 minigene in COS-1/HepG2, morpholino and U7snRNA rescue, and patient-derived megakaryocyte FV restoration

    PMID:24085767

    Open questions at the time
    • Therapeutic delivery and durability in vivo not addressed
    • Generalizability to other intronic variants untested
  9. 2014 High

    Showed a synonymous coding variant can cause FV deficiency by activating a cryptic splice site and disrupting an exonic splicing enhancer, broadening the mutational mechanisms producing non-functional, poorly secreted FV.

    Evidence F5 sequencing, mRNA analysis, minigene in COS-1, recombinant mutant protein expression, and morpholino rescue

    PMID:25470420

    Open questions at the time
    • Quantitative contribution of each defect (secretion vs function) not separated
    • In vivo correction not demonstrated
  10. 2015 High

    Defined a gain-of-function bleeding mechanism: aberrant splicing produces a shortened FV that binds and stabilizes TFPI, suppressing thrombin generation.

    Evidence Whole exome sequencing, thrombin generation, TFPI level and binding assays, and cDNA splicing analysis (FV Amsterdam)

    PMID:25634741

    Open questions at the time
    • Structural basis of FV-TFPI binding not resolved at this stage
    • Protein S contribution not yet incorporated
  11. 2021 High

    Demonstrated that cis-acting sequences in F5 exon 13 regulate alternative splicing to FV-Short, and that deletions enhancing this splicing elevate TFPIα and cause severe bleeding.

    Evidence Patient plasma TFPI quantification, F5 cDNA sequencing, minigene heterologous expression, and FV isoform Western blotting (F5-Atlanta)

    PMID:33773040

    Open questions at the time
    • Trans-acting factors controlling exon 13 splicing not identified
    • Normal physiological regulation of FV-Short levels undefined
  12. 2021 High

    Revealed a paradoxical phenotype where a C2-domain conformational mutation impairs secretion yet abolishes APC cofactor activity and confers APC resistance, producing thrombosis despite severe FV deficiency.

    Evidence Patient thrombin generation, recombinant FV Besançon (Ala2086Asp) prothrombinase and APC inactivation assays, and C2-domain modeling

    PMID:33605529

    Open questions at the time
    • Structural validation of the proposed closed C2 conformation lacking
    • In vivo bleeding/thrombosis balance not fully characterized
  13. 2023 High

    Resolved MCFD2 as the primary cargo-binding subunit with LMAN1 acting as a shuttling carrier, and showed the complex is not rate-limiting for FV/FVIII secretion.

    Evidence LMAN1/MCFD2-deficient HEK293T, HepG2, HCT116 cells with secretion and wild-type/mutant rescue experiments

    PMID:36490287

    Open questions at the time
    • Direct FV-MCFD2 binding interface not structurally defined
    • Identity of the rate-limiting secretion step unknown
  14. 2023 High

    Established the trimolecular FV-Short/TFPIα/protein S complex as a synergistic FXa inhibitor and quantified its ~10-fold elevation in East Texas disorder, defining an anticoagulant threshold whose dysregulation causes bleeding.

    Evidence Biochemical reconstitution of the complex, FXa inhibition assays, and circulating complex quantification in patients versus controls

    PMID:36746318

    Open questions at the time
    • Structural architecture of the trimolecular complex not solved
    • Physiological setpoint and regulation of complex abundance unclear

Open questions

Synthesis pass · forward-looking unresolved questions
  • How FV's procofactor, APC-cofactor, and FV-Short/TFPIα anticoagulant roles are balanced in vivo and how exon 13 splicing is normally regulated remain unresolved.
  • No high-resolution structure of FVa or the FV-Short/TFPIα/protein S complex in the corpus
  • Trans-acting regulators of FV-Short splicing unidentified
  • Tissue-level integration of FV's opposing pro- and anticoagulant functions undefined

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0098772 molecular function regulator activity 4 GO:0140313 molecular sequestering activity 3 GO:0008289 lipid binding 1
Localization
GO:0005576 extracellular region 3 GO:0005783 endoplasmic reticulum 3
Pathway
R-HSA-109582 Hemostasis 3 R-HSA-9609507 Protein localization 3
Partners
F10F2LMAN1MCFD2PROS1TFPI
Complex memberships
FV-Short/TFPIα/protein S complexLMAN1-MCFD2 cargo receptor complexprothrombinase complex

Evidence

Reading pass · 15 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2002 FV Cambridge (Arg306Thr) and FV Hong Kong (Arg306Gly) both lack APC cleavage at Arg306, resulting in partial FVa inactivation in the absence of protein S, but near-complete inactivation in the presence of protein S due to stimulation of cleavage at Arg679. Both variants also show slightly impaired APC cofactor activity in FVIIIa degradation. Recombinant expression of FV mutants, purified protein functional assays (APC-mediated inactivation curves, prothrombinase assay, FVIIIa degradation assay) with and without protein S Blood High 12091344
1996 Factor V Leiden (FV:Q506, Arg506Gln) is activated normally by thrombin or factor Xa, but its impaired inactivation by activated protein C (APC) results in lifelong hypercoagulability. Intact FV also functions as an APC cofactor with protein S in the regulation of FVIIIa in the Xase complex. Biochemical characterization of purified mutant FV, functional coagulation assays, APC resistance testing in patient and reconstituted plasma Annals of hematology High 8624369
2000 Mutations in the R2 allele of the F5 gene (including A6755G, Ala2194Gly in the C2 domain) are associated with a relative increase in the more thrombogenic, more glycosylated FV isoform (FV1) compared with the less glycosylated FV2, suggesting that genetically determined differential glycosylation of FV alters the FV1/FV2 ratio. Molecular genetics sequencing, chromatographic separation of FV isoforms, quantitative FV1/FV2 ratio analysis in homozygous R2 carriers vs controls Thrombosis and haemostasis Medium 10744138
2003 FV Leiden increases thrombin generation in the context of severe FVII deficiency, particularly in the presence of APC, by resisting APC-mediated FVa inactivation. This was confirmed in FV-FVII doubly deficient plasma reconstituted with purified normal FV or FV Leiden. Thrombin generation assays in patient plasma and in doubly deficient plasma reconstituted with purified FV variants; FXa and FVa generation measurements with and without APC Blood High 12881304
2009 The LMAN1-MCFD2 protein complex functions as a cargo receptor for ER-to-Golgi transport of coagulation FV and FVIII. MCFD2 is an EF-hand domain protein forming a calcium-dependent heteromeric complex with LMAN1; missense mutations in MCFD2 EF-hand domains abolish LMAN1 interaction and cause combined FV+FVIII deficiency. The B domain of FVIII may be important in mediating interaction with the complex. Genetic identification of LMAN1 and MCFD2 mutations in F5F8D patients; co-immunoprecipitation; cell biological trafficking assays; mutation analysis British journal of haematology High 19183188
2011 LMAN1-deficient mice show ~50% reduction in plasma FV, plasma FVIII, and platelet FV levels, confirming LMAN1's role as a cargo receptor for ER-to-Golgi transport of FV and FVIII. LMAN1 deficiency causes ER distension and accumulation of α1-antitrypsin and GRP78 in hepatocytes, but cathepsin C and Z levels are unaffected. LMAN1 deficiency had no effect on COPII vesicle formation in vitro. Lman1 knockout mouse generation; plasma FV and FVIII quantification; platelet FV measurement; liver ultrastructure analysis; in vitro COPII vesicle budding assay; Western blot Blood High 21795745
2003 FV New Brunswick (Ala221Val) does not impair FV synthesis, secretion, or procoagulant FXa cofactor function, nor does it affect APC-mediated inactivation of FVa. Instead, the mutation reduces stability of the activated FVa heterodimer by increasing the rate of light and heavy chain dissociation at 37°C, which explains the FV deficiency phenotype. Recombinant expression of Ala221Val and control mutants (Ala221Gly, Glu275Gln, Cys220Ala/Cys301Ala); pulse-chase experiments; ELISA; plasma clotting assays; purified prothrombinase assays; thermal stability assays Blood High 12714495
2015 A novel gain-of-function mutation in F5 (c.C2588G) causes aberrant splicing producing a shortened FV protein (FV Amsterdam, missing 623 amino acids from the B domain) that binds tissue factor pathway inhibitor (TFPI), prolonging its half-life and increasing circulating TFPI levels, leading to severely reduced thrombin generation and a bleeding phenotype. Whole exome sequencing; thrombin generation assay; TFPI level measurement; F5 cDNA splicing analysis; TFPI binding assay Blood High 25634741
2021 A novel 832 bp deletion within F5 exon 13 (F5-Atlanta) enhances alternative splicing at the East Texas (ET) splice sites, producing FV-Short, an isoform with a truncated B domain that binds TFPIα with high affinity, thereby elevating circulating TFPIα levels and causing severe bleeding. This demonstrates that cis-acting regulatory sequences within F5 exon 13 control alternative splicing and regulate circulating FV-Short/TFPIα levels. Patient plasma TFPI quantification; F5 cDNA sequencing; minigene heterologous expression in mammalian cells; FV isoform Western blotting Journal of thrombosis and haemostasis High 33773040
2023 FV-Short, a naturally occurring splice isoform of FV with a truncated B domain, forms a high-affinity trimolecular complex with TFPIα and protein S. FV-Short and protein S synergistically stimulate the FXa-inhibitory activity of TFPIα. In East Texas bleeding disorder, the concentration of the FV-Short/protein S/TFPIα complex is increased ~10-fold above normal (~0.2 nM), providing an elevated anticoagulant threshold and causing bleeding. Biochemical characterization of FV-Short/TFPIα/protein S complex; FXa inhibition assays; quantification of circulating complex in East Texas patients vs controls Journal of thrombosis and haemostasis High 36746318
2016 FV Bonn (Ala512Val) causes APC resistance by slowing APC-mediated cleavage at Arg506 (the primary APC cleavage site), with protein S largely restoring inactivation. FV Bonn also shows ~24% reduced APC cofactor activity in FVIIIa inactivation and enhanced procoagulant activity (increased activation rate, possibly enhanced interaction with activated FX) in the absence of APC. In silico modeling places Ala512 in the same loop as Arg506. Recombinant FV Bonn expression; APTT-based APC sensitivity ratio; thrombin generation-based APC sensitivity ratio; purified FVa inactivation kinetics with/without protein S; FVIIIa cofactor activity assay; in silico structural modeling Journal of thrombosis and haemostasis High 27090446
2014 An apparently synonymous F5 variant (c.1281C>G in exon 8) causes FV deficiency by activating a cryptic donor splice site and abolishing an exonic splicing enhancer, producing aberrantly spliced mRNA lacking the last 18 nucleotides of exon 8. The resulting mutant FV protein (lacking amino acids 427–432) is poorly secreted and non-functional. A mutation-specific morpholino antisense oligonucleotide partially corrected the splicing defect. F5 gene sequencing; mRNA analysis; bioinformatics splice-site prediction; F5 minigene model in COS-1 cells; morpholino antisense rescue; COS-1 cell expression of mutant protein Haemophilia High 25470420
2013 A deep-intronic F5 mutation (c.1296+268A>G) activates a cryptic donor splice site causing severe FV deficiency. Mutation-specific antisense morpholino oligonucleotides (MO) and engineered U7 small nuclear RNA constructs dose-dependently restored correct F5 mRNA splicing in COS-1 and HepG2 cells transfected with a F5 minigene, and in patient-derived megakaryocytes ex vivo, where FV protein expression was restored. F5 minigene transfection in COS-1 and HepG2 cells; antisense morpholino and U7snRNA rescue experiments; patient-derived megakaryocyte differentiation from circulating progenitors; immunofluorescence for FV protein Blood High 24085767
2023 MCFD2 is the primary cargo-binding subunit of the LMAN1-MCFD2 complex for FV and FVIII ER-to-Golgi transport: LMAN1 carbohydrate-binding mutants can still partially rescue FV/FVIII secretion, whereas overexpression of MCFD2 alone (wild-type or mutant) rescues FV/FVIII secretion in LMAN1-deficient cells, indicating LMAN1 serves primarily as a shuttling carrier for MCFD2. Overexpression of both proteins together does not further increase FV/FVIII secretion, indicating the LMAN1-MCFD2 complex is not rate-limiting for ER-Golgi transport of FV/FVIII. LMAN1- and MCFD2-deficient cell lines (HEK293T, HepG2, HCT116); FV/FVIII secretion assays; rescue experiments with wild-type and mutant LMAN1/MCFD2 overexpression; functional pathogenicity assays for missense mutations Blood advances High 36490287
2021 FV Besançon (Ala2086Asp) favors a 'closed conformation' of the C2 domain, impairing FV(a) binding to phospholipids. The mutation causes severe FV deficiency by greatly reducing FV secretion. Residual FVa Besançon shows slightly unfavorable prothrombinase kinetics but markedly reduced APC-mediated inactivation (in the presence of protein S), and completely abolished APC cofactor activity, leading to a paradoxical hypercoagulable/thrombotic state despite severe FV deficiency. Thrombin generation in platelet-rich plasma was higher than in controls and extremely APC resistant. Patient plasma thrombin generation (platelet-poor and platelet-rich); FV:C, FV:Ag, and TFPI assays; F5 sequencing; recombinant FVBesançon expression in COS cells; prothrombinase kinetics; APC-mediated FVa inactivation assay; in silico C2 domain modeling Journal of thrombosis and haemostasis High 33605529

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
1988 Assembly of a functional immunoglobulin Fv fragment in Escherichia coli. Science (New York, N.Y.) 796 3285470
1990 A comparison of strategies to stabilize immunoglobulin Fv-fragments. Biochemistry 429 2110478
1990 Small rearrangements in structures of Fv and Fab fragments of antibody D1.3 on antigen binding. Nature 249 2215663
1999 Activated protein C resistance (FV:Q506) and pregnancy. Thrombosis and haemostasis 178 10235434
2000 Structural dynamics of green fluorescent protein alone and fused with a single chain Fv protein. The Journal of biological chemistry 167 10748019
2006 Isolation of anti-CD22 Fv with high affinity by Fv display on human cells. Proceedings of the National Academy of Sciences of the United States of America 140 16763048
2000 Chimeric Fv-zeta or Fv-epsilon receptors are not sufficient to induce activation or cytokine production in peripheral T cells. Blood 134 10961908
1976 Effect of Fv-1 gene product on proviral DNA formation and integration in cells infected with murine leukemia viruses. Proceedings of the National Academy of Sciences of the United States of America 134 181748
2002 Gene therapy for cancer using single-chain Fv fragments specific for 4-1BB. Nature medicine 126 11927939
1996 Engineering antibody Fv fragments for cancer detection and therapy: disulfide-stabilized Fv fragments. Nature biotechnology 122 9631086
2001 Expression of single-chain Fv-Fc fusions in Pichia pastoris. Journal of immunological methods 107 11292488
2008 Selectivity for grasp in local field potential and single neuron activity recorded simultaneously from M1 and F5 in the awake macaque monkey. The Journal of neuroscience : the official journal of the Society for Neuroscience 93 18945904
1989 Fv-4 resistance gene: a truncated endogenous murine leukemia virus with ecotropic interference properties. Journal of virology 92 2555565
1980 Synthesis and circularization of N- and B-tropic retroviral DNA Fv-1 permissive and restrictive mouse cells. Proceedings of the National Academy of Sciences of the United States of America 92 6248878
2002 Functional characterization of recombinant FV Hong Kong and FV Cambridge. Blood 88 12091344
1991 Single-chain immunotoxins directed at the human transferrin receptor containing Pseudomonas exotoxin A or diphtheria toxin: anti-TFR(Fv)-PE40 and DT388-anti-TFR(Fv). Molecular and cellular biology 79 2005905
2010 Recombinant Fv-Hsp70 protein mediates neuroprotection after focal cerebral ischemia in rats. Stroke 67 20075343
2015 A novel mutation in the F5 gene (factor V Amsterdam) associated with bleeding independent of factor V procoagulant function. Blood 61 25634741
1996 Resistance to activated protein C, the FV:Q506 allele, and venous thrombosis. Annals of hematology 57 8624369
1994 Sequencing and modeling of anti-DNA immunoglobulin Fv domains. Comparison with crystal structures. The Journal of biological chemistry 57 8106407
1999 Domain interactions in antibody Fv and scFv fragments: effects on unfolding kinetics and equilibria. FEBS letters 55 10622716
2000 Mutations in the R2 FV gene affect the ratio between the two FV isoforms in plasma. Thrombosis and haemostasis 53 10744138
1999 Expression and characterization of bispecific single-chain Fv fragments produced in transgenic plants. European journal of biochemistry 53 10411643
1993 Transgenic Fv-4 mice resistant to Friend virus. Journal of virology 51 8510219
1994 Mammalian expression and secretion of functional single-chain Fv molecules. The Journal of biological chemistry 49 7929344
2009 Recent developments in the understanding of the combined deficiency of FV and FVIII. British journal of haematology 48 19183188
1993 A bacterially expressed single-chain Fv construct from the 2B4 T-cell receptor. Proceedings of the National Academy of Sciences of the United States of America 47 8387198
1968 Deoxyribonucleic acid synthesis in FV-3-infected mammalian cells. Journal of virology 46 5749374
2011 Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of α1-antitrypsin. Blood 44 21795745
1999 Fluobodies: green fluorescent single-chain Fv fusion proteins. Journal of immunological methods 44 10594359
1995 Disulfide stabilization of antibody Fv: computer predictions and experimental evaluation. Protein engineering 43 8869646
2003 Recurrent pregnancy loss and its relation to FV Leiden, FII G20210A and polymorphisms of plasminogen activator and plasminogen activator inhibitor. Pathophysiology of haemostasis and thrombosis 42 15170393
1994 Structural and dynamic properties of the Fv fragment and the single-chain Fv fragment of an antibody in solution investigated by heteronuclear three-dimensional NMR spectroscopy. Biochemistry 42 8136365
1993 Protein Fv produced during vital hepatitis is a novel activator of human basophils and mast cells. Journal of immunology (Baltimore, Md. : 1950) 42 7693815
1993 Synthesis and expression of a DNA encoding the Fv domain of an anti-lysozyme monoclonal antibody, HyHEL10, in Streptomyces lividans. Gene 42 8335251
1984 A cell membrane "gp70" associated with Fv-4 gene: immunological characterization, and tissue and strain distribution. Virology 42 6199893
2008 Combined FV and FVIII deficiency. Haemophilia : the official journal of the World Federation of Hemophilia 41 19141160
1985 Analysis of wild-derived mice for Fv-1 and Fv-2 murine leukemia virus restriction loci: a novel wild mouse Fv-1 allele responsible for lack of host range restriction. Journal of virology 41 2991555
1997 Molecular cloning, expression, and characterization of a functional single-chain Fv antibody to the mycotoxin zearalenone. Applied and environmental microbiology 39 8979354
1993 Cloning and expression of an autoimmune DNA-binding single chain Fv. Only the heavy chain is required for binding. Molecular immunology 38 8321248
1981 Fv-1 host restriction of Friend leukemia virus: analysis of unintegrated proviral DNA. Journal of virology 38 7288927
2014 Increased coagulation activity and genetic polymorphisms in the F5, F10 and EPCR genes are associated with breast cancer: a case-control study. BMC cancer 37 25407022
2013 Clostridium botulinum strain Af84 contains three neurotoxin gene clusters: bont/A2, bont/F4 and bont/F5. PloS one 37 23637798
2003 Coinheritance of Factor V (FV) Leiden enhances thrombin formation and is associated with a mild bleeding phenotype in patients homozygous for the FVII 9726+5G>A (FVII Lazio) mutation. Blood 37 12881304
1997 Direct T cell activation by chimeric single chain Fv-Syk promotes Syk-Cbl association and Cbl phosphorylation. The Journal of biological chemistry 35 9079685
1993 Bacterial expression of a single-chain Fv fragment which efficiently protects the acetylcholine receptor against antigenic modulation caused by myasthenic antibodies. European journal of immunology 35 8344344
2004 Immunosuppressive properties of anti-CD3 single-chain Fv and diabody. Journal of immunological methods 33 14871540
2000 Location and extent of deep vein thrombosis in patients with and without FV:R 506Q mutation. Thrombosis and haemostasis 33 10823255
1999 Intrabody construction and expression. II. A synthetic catalytic Fv fragment. Journal of molecular biology 33 10518948
1996 Single-chain Fv radioimmunotargeting. The quarterly journal of nuclear medicine : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR) 31 8961808
1994 On the interaction between single chain Fv antibodies and bacterial immunoglobulin-binding proteins. Journal of immunological methods 30 7822821
1993 Expression, purification and characterization of B72.3 Fv fragments. The Biochemical journal 30 8457200
2011 Single-chain Fv-based anti-HIV proteins: potential and limitations. Journal of virology 29 22013046
1985 Cellular site and mode of Fv-2 gene action. Cell 29 3855388
2003 Activated protein C resistance (FV(Leiden)) and thrombosis: factor V mutations causing hypercoagulable states. Hematology/oncology clinics of North America 28 12627662
2009 Isolation of bovine intestinal Lactobacillus plantarum and Pediococcus acidilactici with inhibitory activity against Escherichia coli O157 and F5. Journal of applied microbiology 27 19200307
1996 Use of nanogold- and fluorescent-labeled antibody Fv fragments in immunocytochemistry. The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 27 8648079
2022 The AP2/ERF transcription factor SlERF.F5 functions in leaf senescence in tomato. Plant cell reports 26 35238951
2003 Factor V New Brunswick: Ala221Val associated with FV deficiency reproduced in vitro and functionally characterized. Blood 25 12714495
1995 Bacterial expression and refolding of single-chain Fv fragments with C-terminal cysteines. Cell biophysics 25 8521451
2021 Severe thrombophilia in a factor V-deficient patient homozygous for the Ala2086Asp mutation (FV Besançon). Journal of thrombosis and haemostasis : JTH 22 33605529
2021 F5-Atlanta: A novel mutation in F5 associated with enhanced East Texas splicing and FV-short production. Journal of thrombosis and haemostasis : JTH 22 33773040
2016 Association of gene polymorphisms of FV, FII, MTHFR, SERPINE1, CTLA4, IL10, and TNFalpha with pre-eclampsia in Chinese women. Inflammation research : official journal of the European Histamine Research Society ... [et al.] 22 27233255
2013 Novel immunocytokine IL12-SS1 (Fv) inhibits mesothelioma tumor growth in nude mice. PloS one 22 24260587
1999 Synthesis of Rh Fv phage-antibodies using VH and VL germline genes. British journal of haematology 22 10354152
2023 Natural anticoagulant discovery, the gift that keeps on giving: finding FV-Short. Journal of thrombosis and haemostasis : JTH 21 36746318
1981 Fate of unintegrated viral DNA in Fv-1 permissive and resistant mouse cells infected with murine leukemia virus. Journal of virology 20 6938734
2002 Production and characterization of an anti-idiotypic single chain Fv that recognizes an anti-DNA antibody. Immunological investigations 19 12472180
1997 Expression and characterization of recombinant single-chain Fv and Fv fragments derived from a set of catalytic antibodies. Molecular immunology 19 9464525
1995 Use of antibody fragments (Fv) in immunocytochemistry. The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 19 7769231
1978 Transfection of Fv-1 permissive and restrictive mouse cells with integrated DNA of murine leukemia viruses. Proceedings of the National Academy of Sciences of the United States of America 19 206900
2023 Immunoaffinity biosensors for the detection of SARS-CoV-1 using screened Fv-antibodies from an autodisplayed Fv-antibody library. Biosensors & bioelectronics 18 37301177
2019 F5-Peptide and mTORC1/rpS6 Effectively Enhance BTB Transport Function in the Testis-Lesson From the Adjudin Model. Endocrinology 17 31157869
2016 Identification and functional characterization of a novel F5 mutation (Ala512Val, FVB onn ) associated with activated protein C resistance. Journal of thrombosis and haemostasis : JTH 17 27090446
2014 Three enzymatically active neurotoxins of Clostridium botulinum strain Af84: BoNT/A2, /F4, and /F5. Analytical chemistry 17 24605815
2014 Characterization of an apparently synonymous F5 mutation causing aberrant splicing and factor V deficiency. Haemophilia : the official journal of the World Federation of Hemophilia 17 25470420
2010 Expression and purification of an anti-clenbuterol single chain Fv antibody in Escherichia coli. Protein expression and purification 17 20206697
2008 The joint association between F5 gene polymorphisms and maternal smoking during pregnancy on preterm delivery. Human genetics 17 19020903
2020 Exome Sequencing Reveals Common and Rare Variants in F5 Associated With ACE Inhibitor and Angiotensin Receptor Blocker-Induced Angioedema. Clinical pharmacology and therapeutics 16 32496628
2019 MTHFR and F5 genetic variations have association with preeclampsia in Pakistani patients: a case control study. BMC medical genetics 16 31646966
2013 Antisense-based RNA therapy of factor V deficiency: in vitro and ex vivo rescue of a F5 deep-intronic splicing mutation. Blood 15 24085767
2000 Construction and structural modeling of a single-chain Fv-asparaginase fusion protein resistant to proteolysis. Biotechnology and bioengineering 15 11005928
2021 Fluorescein and Rhodamine B-Binding Domains from Autodisplayed Fv-Antibody Library. Bioconjugate chemistry 14 34617729
1996 Construction and expression of bi-functional proteins of single-chain Fv with effector domains. Journal of biochemistry 14 8889803
1996 Characterization of T cell receptor single-chain Fv fragments secreted by myeloma cells. European journal of immunology 14 8898954
2023 Monoamine Oxidase-A (MAO-A) Inhibitors Screened from the Autodisplayed Fv-Antibody Library. ACS pharmacology & translational science 13 38230273
2022 De novo design and Rosetta-based assessment of high-affinity antibody variable regions (Fv) against the SARS-CoV-2 spike receptor binding domain (RBD). Proteins 13 36111441
2021 FV-429 enhances the efficacy of paclitaxel in NSCLC by reprogramming HIF-1α-modulated FattyAcid metabolism. Chemico-biological interactions 13 34648812
2018 Single-chain Fv Antibodies for Targeting Neurodegenerative Diseases. CNS & neurological disorders drug targets 13 29546836
2013 Germ-line MTHFR C677T, FV H1299R and PAI-1 5G/4G variations in breast carcinoma. Asian Pacific journal of cancer prevention : APJCP 13 23803051
2001 Single chain Fv: a ligand in receptor-mediated gene delivery. Gene therapy 13 11320404
2001 Antibody engineering for targeted therapy of cancer: recombinant Fv-immunotoxins. Current pharmaceutical biotechnology 13 11482347
2014 Clostridium botulinum strains producing BoNT/F4 or BoNT/F5. Applied and environmental microbiology 12 24632257
2023 The clinical relevance of the adhesion G protein-coupled receptor F5 for human diseases and cancers. Biochimica et biophysica acta. Molecular basis of disease 11 36878303
2017 Alcoholic Fractions F5 and F6 from Withania somnifera Leaves Show a Potent Antileishmanial and Immunomodulatory Activities to Control Experimental Visceral Leishmaniasis. Frontiers in medicine 11 28553635
2003 Generation and characterization of recombinant single chain Fv antibody that recognizes platelet glycoprotein Ibalpha. Thrombosis research 11 12706643
2023 Separate roles of LMAN1 and MCFD2 in ER-to-Golgi trafficking of FV and FVIII. Blood advances 10 36490287
2020 Genome-based classification of Acidihalobacter prosperus F5 (=DSM 105917=JCM 32255) as Acidihalobacter yilgarnensis sp. nov. International journal of systematic and evolutionary microbiology 10 33112221
2019 Biophysical characterization and single-chain Fv construction of a neutralizing antibody to measles virus. The FEBS journal 10 31287622
2007 Protein Fv: an endogenous immunoglobulin superantigen and superallergen. Chemical immunology and allergy 10 17369700

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