VWF

Synonyms

F8VWF, von Willebrand antigen II, VWF, Von Willebrand Factor, Coagulation Factor VIII VWF, VWD

Description

VWF (Von willebrand factor) is important in the maintenance of hemostasis. It promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. It also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma.

KO Status

RenMab: F0

Drug Information

Launched drugs: 2
Drug in clinical trials: 1
Latest Research Phase: Approved

Drug Name

Code

Phase

Company

Indications

Clinical Trials

Defibrotide sodium

JZP-381

Approved

Jazz Pharmaceuticals, Gentium Srl

Lymphoma, Large B-Cell, Diffuse, Neurotoxicity Syndromes, Hepatic Veno-Occlusive Disease

Caplacizumab

ALX-0081, ALX-0681

Approved

Ablynx

Angina, Unstable, Purpura, Thrombotic Thrombocytopenic, Acquired thrombotic thrombocytopenic purpura

Apadamtase alfa

TAK-755, SHP-655, rADAMTS-13, BAX-930

Phase 3 Clinical

Kaketsuken

Purpura, Thrombotic Thrombocytopenic, Acquired thrombotic thrombocytopenic purpura, Anemia, Sickle Cell

BT-200

BT-200

Phase 1 Clinical

Band Therapeutics

Atherosclerosis, Coronary Artery Disease, Intracranial Arteriosclerosis, Stroke

DTRI-031

DTRI-031

Preclinical

Basking Biosciences Inc

Stroke

TAGX-0004

TAGX-0004

Preclinical

TagCyx Biotechnologies

von Willebrand Diseases

Saratin

Pending

Merck & Co Inc

Thrombosis

GRC-600

GRC-600, CHR-1201

Pending

Chromos Molecular Systems

Stroke, Acute Coronary Syndrome, Thrombosis

AJW-200

AJW-200

Pending

Ajinomoto Co Inc

Thrombosis

Egaptivon pegol

ARC-1779

Discontinued

Archemix

Intracranial Embolism, von Willebrand Diseases, Purpura, Thrombotic Thrombocytopenic, Thrombotic Microangiopathies, von Willebrand Disease, Type 2, Hemolytic-Uremic Syndrome, Carotid Stenosis, Acquired thrombotic thrombocytopenic purpura, Thrombosis

References


Title

Authors

Source

Type IIB von Willebrand's disease: gene mutations and clinical presentation in nine families from Denmark, Germany and Sweden

Donner M., Kristoffersson A.-C., Lenk H., Scheibel E., Dahlback B., Nilsson I.M., Holmberg L.,

Br. J. Haematol. 82:58-65(1992)

Defects in type IIA von Willebrand disease: a cysteine 509 to arginine substitution in the mature von Willebrand factor disrupts a disulphide loop involved in the interaction with platelet glycoprotein Ib-IX

Lavergne J.-M., de Paillette L., Bahnak B.R., Ribba A.-S., Fressinaud E., Meyer D., Pietu G.,

Br. J. Haematol. 82:66-72(1992)

Characterization of recombinant von Willebrand factor corresponding to mutations in type IIA and type IIB von Willebrand disease

Ribba A.S., Voorberg J., Meyer D., Pannekoek H., Pietu G.,

J. Biol. Chem. 267:23209-23215(1992)

von Willebrand disease type B: a missense mutation selectively abolishes ristocetin-induced von Willebrand factor binding to platelet glycoprotein Ib

Rabinowitz I., Tuley E.A., Mancuso D.J., Randi A.M., Firkin B.G., Howard M.A., Sadler J.E.,

Proc. Natl. Acad. Sci. U.S.A. 89:9846-9849(1992)

Identification of three candidate mutations causing type IIA von Willebrand disease using a rapid, nonradioactive, allele-specific hybridization method

Inbal A., Englender T., Kornbrot N., Randi A.M., Castaman G., Mannucci P.M., Sadler J.E.,

Blood 82:830-836(1993)

Substitution of cysteine for phenylalanine 751 in mature von Willebrand factor is a novel candidate mutation in a family with type IIA von Willebrand disease

Gaucher C., Hanss M., Dechavanne M., Mazurier C.,

Br. J. Haematol. 83:94-99(1993)

Two new candidate mutations in type IIA von Willebrand's disease (Arg834-->Gly, Gly846-->Arg) and one polymorphism (Tyr821-->Cys) in the A2 region of the von Willebrand factor

Donner M., Kristoffersson A.C., Berntorp E., Scheibel E., Thorsen S., Dahlback B., Nilsson I.M., Holmberg L.,

Eur. J. Haematol. 51:38-44(1993)

Type IIB mutation His-505-->Asp implicates a new segment in the control of von Willebrand factor binding to platelet glycoprotein Ib

Rabinowitz I., Randi A.M., Shindler K.S., Tuley E.A., Rustagi P.K., Sadler J.E.,

J. Biol. Chem. 268:20497-20501(1993)

von Willebrand factor mutation enhancing interaction with platelets in patients with normal multimeric structure

Holmberg L., Dent J.A., Schneppenheim R., Budde U., Ware J., Ruggeri Z.M.,

J. Clin. Invest. 91:2169-2177(1993)

The molecular defect in type IIB von Willebrand disease. Identification of four potential missense mutations within the putative GpIb binding domain

Cooney K.A., Nichols W.C., Bruck M.E., Bahou W.F., Shapiro A.D., Bowie E.J.W., Gralnick H.R., Ginsburg D.,

J. Clin. Invest. 87:1227-1233(1991)