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Protein / target

von Willebrand factor

Encoded byVWFP04275Homo sapiensSwiss-Prot
Clinically validated
Therapeutic maturity
3
Approved medicines
Open Targets target-level
View by indication →
10
Clinical trials
Small-molecule tractable
Druggability
Med-Quality Pocket

Protein at a glance

Biological role

Extracellular matrix structural constituent

Strongest disease association

von Willebrand disease 2

Via encoding gene VWF · Genetic evidence · score 0.98

Therapeutic position

Established drug target

Antibodies

Research activity

Emerging research

2 papers · latest 2024

Derived from structured UniProt, Open Targets and literature data on this page.

Protein profile

UniProt 2026_02

Canonical identity and biological annotation from UniProt.

Function overview

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.

View complete UniProt function annotation

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. 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

Subcellular location

SecretedSecreted, extracellular space, extracellular matrix
Domains and Gene Ontology detail (37)

Domains & features

VWFD 1TIL 1VWFD 2TIL 2TIL 3VWFD 3TIL 4VWFA 1; binding site for platelet glycoprotein IbVWFA 2VWFA 3; main binding site for collagens type I and IIIVWFD 4VWFC 1VWFC 2VWFC 3CTCK

Gene Ontology

  • Cendoplasmic reticulum
  • Cextracellular exosome
  • Cextracellular matrix
  • Cextracellular region
  • Cextracellular space
  • Cplatelet alpha granule
  • Cplatelet alpha granule lumen
  • CWeibel-Palade body
  • Fcollagen binding
  • Fextracellular matrix structural constituent
  • Fidentical protein binding
  • Fimmunoglobulin binding

2813 aa · 309 kDa · 2 isoforms

Biological roles

Reactome v97

What this protein does, drawn together from its UniProt function, Gene Ontology terms and Reactome pathways.

HaemostasisUniProt · GO · ReactomeCell adhesionUniProt · GOKinase signallingReactome
View supporting evidence

Haemostasis

  • ·Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sit…
  • ·platelet alpha granule
  • ·platelet alpha granule lumen
  • ·blood coagulation

Cell adhesion

  • ·Secreted, extracellular space, extracellular matrix
  • ·extracellular matrix
  • ·extracellular matrix structural constituent
  • ·cell adhesion

Kinase signalling

  • ·Signaling by moderate kinase activity BRAF mutants
  • ·Signaling by high-kinase activity BRAF mutants
View underlying pathways (25)

Concepts derived from UniProt GO Reactome — each badge above shows which sources supported that role.

Interaction neighbourhood

STRING v12.0

Proteins with the strongest functional or physical association. Node size and line weight reflect STRING confidence; hover or select a partner to inspect one association.

F8GP1BASELPITGA2BITGB3GP9FN1GP1BBADAMTS…GP6VWF

10 strongest partners — larger node and heavier line mean higher confidence

Functional and physical associations from STRING v12.0. Only associations with this protein are drawn — partner-to-partner links are not part of this evidence.

Approved medicines with mapped indications

2 medicines · 2 areas

Approved therapies targeting this protein, grouped by what they are approved to treat — the disease-first view of the medicines below. Relationships come from the canonical approved-indication graph (ChEMBL phase-4), the same source as the drug cards.

Thrombosis1 medicine
von Willebrand Diseases1 medicine

3 medicines meet Open Targets' target-level approved-medicine definition; the 2 shown here are those on this page with a canonical approved disease indication in the graph. Approved indications from ChEMBL (phase-4), via the canonical drug→disease graph.

Drugs targeting this protein

2

How approved and investigational drugs engage this protein — mechanism and action type, direct vs complex targeting, and how broadly each acts across other targets. A drug-first view (the section above groups the approved ones disease-first); direct binders with few recorded targets are listed first.

caplacizumab
Narrow target profileApprovedInhibitor

von Willebrand factor inhibitor

Indicated for Thrombosis

Direct interaction with this protein · Only this protein recorded as a target

vonicog alfa
Narrow target profileApprovedExogenous protein

von Willebrand factor exogenous protein

Indicated for von Willebrand Diseases

Direct interaction with this protein · Only this protein recorded as a target

ChEMBL mechanism, action type, target identity and approved indications. Open Targets clinical status.

Translational evidence

Open Targets 26

Why this target matters therapeutically, strongest evidence first. Disease associations are gene-level (via the gene that encodes this protein) and open into the full confidence synthesis; the development universe, tractability and safety annotations are target-level, from Open Targets.

Strongest disease associations · via encoding gene VWF

Gene-level evidence surfaced through the gene VWFthat encodes this protein — not a direct protein–disease relationship. Ranked by Forefront's causal-directness weighting, so genetically- and clinically-evidenced diseases lead over ones that merely share the literature.

von Willebrand disease 2
0.99Well supported

Genetic evidence dominant · Open Targets 0.77

Von Willebrand disease
0.97Well supported

Genetic evidence dominant · Open Targets 0.83

von Willebrand disease (hereditary or acquired)
0.97Well supported

Genetic evidence dominant · Open Targets 0.69

Von Willebrand disease type 2
0.96Well supported

Genetic evidence dominant · Open Targets 0.70

hereditary von Willebrand disease
0.96Well supported

Genetic evidence dominant · Open Targets 0.67

View evidence synthesis (5)
von Willebrand disease 2Well supported
0.99
agreement 0.871.00
Genetic82%Literature10%Animal model9%Genetic literaturedup

Open Targets aggregate 0.77 · 3 independent evidence families · 1 not counted as duplicate

Von Willebrand diseaseWell supported
0.97
agreement 0.881.00
Genetic46%Clinical36%Animal model13%Literature5%Genetic literaturedup

Open Targets aggregate 0.83 · 4 independent evidence families · 1 not counted as duplicate

von Willebrand disease (hereditary or acquired)Well supported
0.97
agreement 0.861.00
Genetic55%Clinical36%Literature9%

Open Targets aggregate 0.69 · 3 independent evidence families

Von Willebrand disease type 2Well supported
0.96
agreement 0.841.00
Genetic87%Animal model10%Literature4%Genetic literaturedup

Open Targets aggregate 0.70 · 3 independent evidence families · 1 not counted as duplicate

hereditary von Willebrand diseaseWell supported
0.96
agreement 0.821.00
Genetic100%Literature0%Genetic literaturedup

Open Targets aggregate 0.67 · 2 independent evidence families · 1 not counted as duplicate

This ranking differs from Open Targets' own: re-weighting moves genetically-evidenced diseases above more heavily co-mentioned ones. The evidence agreement range shows how closely the independent evidence families agree — it is not a statistical confidence interval, and nothing here is fitted to outcome data. Derived from Open Targets evidence types under Forefront weighting; the per-type scores above show the calculation.

Show all associations
Von Willebrand disease0.83
von Willebrand disease 30.80
von Willebrand disease 20.77
Von Willebrand disease type 10.73
von Willebrand disease type 2B0.71
Von Willebrand disease type 20.70
von Willebrand disease 10.69
von Willebrand disease (hereditary or acquired)0.69
hereditary von Willebrand disease0.67
von Willebrand disease type 2A0.67

Drug development

4 compounds recorded · 3 approved · 1 in clinical development

Open Targets' development universe — every compound recorded against the target at any stage, not all approved medicines. Distinct from the 2 drugs that target this protein in Forefront's canonical graph (2 with a mapped approved indication, shown above): these count different sets and are not a subset relation.

View all recorded compounds (4)
EGAPTIVON PEGOLPhase 2
CAPLACIZUMABApproval
VON WILLEBRAND FACTOR HUMANApproval
VONICOG ALFAApproval

Open Targets known-drugs universe. Drug name and highest clinical stage only — the disease relationship is NOT read from this slice (it carries trial-context noise); approved indications come from the canonical graph above.

Tractability

Small moleculesEmerging

Feasibility evidence (med-quality pocket) — no clinical-stage drug of this modality recorded.

AntibodiesStrong

Approved Drug and UniProt loc high conf support this modality.

Protein degradersEmerging

Feasibility evidence (database ubiquitination) — no clinical-stage drug of this modality recorded.

Other modalitiesStrong

Approved Drug support this modality.

View underlying tractability evidence (7)
SM · Med-Quality PocketAB · Approved DrugAB · UniProt loc high confAB · GO CC high confAB · UniProt SigP or TMHMMPR · Database UbiquitinationOC · Approved Drug

Raw Open Targets tractability assessment buckets, by modality.

Clinical trials

10

Trials of drugs that target this protein — reached indirectly through those drugs, so a trial listed here studies the drug, not the protein. Ranked by active status, then clinical phase and recency, and spread across the targeting drugs.

View all trials (6)

ClinicalTrials.gov via the drug-target graph.

What's happening now

3

Recent therapeutic activity around this target — regulatory actions, clinical trials and safety signals for 2 drugs that target this protein, plus publications where such a drug is a genuine subject. Every item is reached indirectly through the drug, not the protein itself; incidental mentions (a paper that merely measures a drug) and press items are excluded.

  1. New publication2020-02-01
    The Therapeutic Potential of Nanobodies.

    BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy · 2020 · 535 citations · Europe PMC · via caplacizumab

  2. Regulatory approval2018-08-31

    Approval: Veyvondi (EMA)

    ema · regulatory · ema · via vonicog alfa

  3. Regulatory approval2018-08-30

    Approval: Cablivi (EMA)

    ema · regulatory · ema · via caplacizumab

Objective event titles are shown unmodified; the event kind and significance line are derived from structured fields. Forefront AttentionEvent stream aggregating Europe PMC Regulatory filings ClinicalTrials.gov.

Research activity

2 papers · to 2024

Papers linked directly to this protein. This is the protein's own literature — descriptor-derived papers are kept separate below.

Most cited

Recent

Europe PMC papers linked directly to this protein.