Peringatan Keamanan

There is limited information regarding the LD50 and overdose of vWF.

Von Willebrand factor human

DB13133

biotech approved investigational

Deskripsi

The human von Willebrand factor (vWF) is a human plasma-derived vWF, an endogenous large multimeric plasma glycoprotein involved in hemostasis. It serves a dual role in hemostasis by mediating platelet adhesion and aggregation at the site of blood vessel injury and stabilizing procoagulant factor VIII (FVIII).A32262 Exogenous sources of vWF are used to restore functional levels of vWF in blood disorders associated with deficient or abnormal blood clotting. The human vWF is used to manage and control bleeding episodes in patients with von Willebrand disease and hemophilia A. It was first approved by the FDA in 2015.L40049 As vWF is normally present in the blood as a stable complex with coagulation factor III, therapeutic vWF products are also available as a combination product with antihemophilic factor human.L1878 A recombinant form of vWF, vonicog alfa, is also available to enhance production and avoid the theoretical risk of pathogen transmission from plasma donors.A32262

Struktur Molekul 2D

Struktur tidak tersedia

Peta Jejaring Molekuler
Legenda: ObatTargetGenEnzim(Panah → menunjukkan arah efek / relasi)TransporterCarrier

Profil Farmakokinetik

Waktu Paruh (Half-Life) In patients with Type 3 von Willebrand disease who were previously treated on-demand with any vWF product prior to study entry, the mean (SD) half-life of recombinant vWF at steady state was 16.5 (4.13) hours. In patients who were previously treated prophylactically with a plasma-derived vWF product, the mean (SD) half-life was 14.1 (6.13) hours.[L40049]
Volume Distribusi The volume of distribution of the human concentrate vWF is 69.7 mL/kg.[A32270]
Klirens (Clearance) In patients with Type 3 von Willebrand disease who were previously treated on-demand with any vWF product prior to study entry, the mean (SD) clearance of recombinant vWF at steady state was 0.04 (0.012) (dL/kg)/h. In patients who were previously treated prophylactically with a plasma-derived vWF product, the mean (SD) clearance was 0.04 (0.014) (dL/kg)/h.[L40049]

Absorpsi

In patients with Type 3 von Willebrand disease who were previously treated on-demand with any vWF product prior to study entry, the mean (SD) Cmax of recombinant vWF at steady state was 86.4 (34.2) IU/dL. In patients who were previously treated prophylactically with a plasma-derived vWF product, the mean (SD) Cmax was 90.6 (33.7) IU/dL.L40049

Metabolisme

ADAMTS13, or von Willebrand factor-cleaving protease, is a disintegrin and metalloprotease that normally cleaves vWF.A32262 Proteolysis of vWF occurs primarily in the cleavage site at domain A2, which is a target domain for ADAMTS13.A32290

Rute Eliminasi

As with endogenous vWF, exogenous sources of vWF are also expected to undergo elimination by the liver and spleen, which take up vWF as part of an active regulatory mechanism.A32266

Interaksi Obat

92 Data
Aminocaproic acid The risk or severity of adverse effects can be increased when Aminocaproic acid is combined with Von Willebrand factor human.
Alpha-1-proteinase inhibitor Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Von Willebrand factor human.
Menadione Menadione may increase the thrombogenic activities of Von Willebrand factor human.
Tranexamic acid Tranexamic acid may increase the thrombogenic activities of Von Willebrand factor human.
Aprotinin Aprotinin may increase the thrombogenic activities of Von Willebrand factor human.
Hydrogen peroxide Hydrogen peroxide may increase the thrombogenic activities of Von Willebrand factor human.
Aminomethylbenzoic acid Aminomethylbenzoic acid may increase the thrombogenic activities of Von Willebrand factor human.
Camostat Camostat may increase the thrombogenic activities of Von Willebrand factor human.
Menadione bisulfite Menadione bisulfite may increase the thrombogenic activities of Von Willebrand factor human.
Monteplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Monteplase.
Lepirudin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Lepirudin.
Bivalirudin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Bivalirudin.
Alteplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Alteplase.
Urokinase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Urokinase.
Reteplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Reteplase.
Anistreplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Anistreplase.
Tenecteplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Tenecteplase.
Abciximab The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Abciximab.
Drotrecogin alfa The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Drotrecogin alfa.
Streptokinase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Streptokinase.
Dicoumarol The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Dicoumarol.
Argatroban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Argatroban.
Ardeparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Ardeparin.
Phenindione The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Phenindione.
Fondaparinux The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Fondaparinux.
Warfarin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Warfarin.
Pentosan polysulfate The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Pentosan polysulfate.
Phenprocoumon The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Phenprocoumon.
Dipyridamole The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Dipyridamole.
Heparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Heparin.
Enoxaparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Enoxaparin.
Epoprostenol The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Epoprostenol.
Acenocoumarol The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Acenocoumarol.
4-hydroxycoumarin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with 4-hydroxycoumarin.
Coumarin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Coumarin.
Ximelagatran The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Ximelagatran.
Desmoteplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Desmoteplase.
Defibrotide The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Defibrotide.
Ancrod The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Ancrod.
Beraprost The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Beraprost.
Prasugrel The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Prasugrel.
Rivaroxaban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Rivaroxaban.
Sulodexide The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Sulodexide.
Idraparinux The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Idraparinux.
Cangrelor The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Cangrelor.
Astaxanthin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Astaxanthin.
Apixaban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Apixaban.
Otamixaban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Otamixaban.
Amediplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Amediplase.
Dabigatran etexilate The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Dabigatran etexilate.
Danaparoid The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Danaparoid.
Dalteparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Dalteparin.
Tinzaparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Tinzaparin.
(R)-warfarin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with (R)-warfarin.
Ethyl biscoumacetate The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Ethyl biscoumacetate.
Nadroparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Nadroparin.
Triflusal The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Triflusal.
Ticagrelor The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Ticagrelor.
Ditazole The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Ditazole.
Vorapaxar The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Vorapaxar.
Edoxaban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Edoxaban.
Sodium citrate The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Sodium citrate.
Dextran The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Dextran.
Bemiparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Bemiparin.
Parnaparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Parnaparin.
Desirudin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Desirudin.
Antithrombin Alfa The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Antithrombin Alfa.
Protein C The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Protein C.
Antithrombin III human The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Antithrombin III human.
Letaxaban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Letaxaban.
Darexaban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Darexaban.
Betrixaban The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Betrixaban.
Nafamostat The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Nafamostat.
Gabexate The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Gabexate.
Fluindione The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Fluindione.
Protein S human The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Protein S human.
Brinase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Brinase.
Clorindione The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Clorindione.
Diphenadione The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Diphenadione.
Tioclomarol The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Tioclomarol.
Melagatran The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Melagatran.
Saruplase The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Saruplase.
(S)-Warfarin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with (S)-Warfarin.
Tocopherylquinone The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Tocopherylquinone.
Dabigatran The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Dabigatran.
Semuloparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Semuloparin.
Troxerutin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Troxerutin.
Edetic acid The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Edetic acid.
Reviparin The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Reviparin.
Dermatan sulfate The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Dermatan sulfate.
SR-123781A The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with SR-123781A.
Limaprost The therapeutic efficacy of Von Willebrand factor human can be decreased when used in combination with Limaprost.

Target Protein

Coagulation factor VIII F8
Collagen alpha-1(I) chain COL1A1
Asialoglycoprotein receptor 1 ASGR1
Prolow-density lipoprotein receptor-related protein 1 LRP1

Referensi & Sumber

Artikel (PubMed)
  • PMID: 19768705
    Clifton JG, Huang F, Kovac S, Yang X, Hixson DC, Josic D: Proteomic characterization of plasma-derived clotting factor VIII-von Willebrand factor concentrates. Electrophoresis. 2009 Oct;30(20):3636-46. doi: 10.1002/elps.200900270.
  • PMID: 21220152
    Klukowska A, Windyga J, Batorova A: Clinical efficacy of a novel VWF-containing FVIII concentrate, Wilate((R)), in the prophylaxis and treatment of bleeding episodes in previously treated haemophilia A patients. Thromb Res. 2011 Mar;127(3):247-53. doi: 10.1016/j.thromres.2010.11.030. Epub 2011 Jan 8.
  • PMID: 25712991
    Lenting PJ, Christophe OD, Denis CV: von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends. Blood. 2015 Mar 26;125(13):2019-28. doi: 10.1182/blood-2014-06-528406. Epub 2015 Feb 23.
  • PMID: 26239086
    Gill JC, Castaman G, Windyga J, Kouides P, Ragni M, Leebeek FW, Obermann-Slupetzky O, Chapman M, Fritsch S, Pavlova BG, Presch I, Ewenstein B: Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015 Oct 22;126(17):2038-46. doi: 10.1182/blood-2015-02-629873. Epub 2015 Aug 3.
  • PMID: 18263586
    Chung MC, Popova TG, Jorgensen SC, Dong L, Chandhoke V, Bailey CL, Popov SG: Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy. J Biol Chem. 2008 Apr 11;283(15):9531-42. doi: 10.1074/jbc.M705871200. Epub 2008 Feb 8.

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