von Willebrand disease-specific defects and proteomic signatures in endothelial colony–forming cells
Lower plasma VWF was linked to reduced production and secretion by ECFCs obtained from patients. Furthermore, nonresponse to DDAVP in some patients was explained by VWF retention in the endoplasmic reticulum. The correlation between functional aspects of ECFCs and their quantitative polymerase chain reaction and proteome profiles yielded potential targets for further research.
Authors
- Stijn A. Groten (ORCID: https://orcid.org/0000-0002-9438-9939)
- Frank W.G. Leebeek (ORCID: https://orcid.org/0000-0001-5677-1371)
- Petra E. Bürgisser (ORCID: https://orcid.org/0000-0002-7832-8028)
- Jeroen C.J. Eikenboom (ORCID: https://orcid.org/0000-0002-3268-5759)
- Iris van Moort (ORCID: https://orcid.org/0000-0002-0248-4710)
- Sebastiaan Laan (ORCID: https://orcid.org/0000-0001-9206-8712)
- Ruben Bierings (ORCID: https://orcid.org/0000-0002-1205-9689)
- Richard Dirven (ORCID: https://orcid.org/0000-0001-9626-9925)
- Maartje van den Biggelaar
Institutions
- Leiden University Medical Center (NL)
- Sanquin (NL)
- Erasmus University Rotterdam (NL)
Publication Details
- Journal
- Journal of Thrombosis and Haemostasis
- Published
- 2025-05-06
- DOI
- https://doi.org/10.1016/j.jtha.2025.04.024
- Citations
- 4
- Primary Topic
- Platelet Disorders and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 7.08
Funders
- CSL Behring
- Takeda Pharmaceutical Company
- Landsteiner Foundation for Blood Transfusion Research
- Amsterdam University Medical Centers
- Universiteit Leiden
- Radboud Universiteit
- Nederlandse Organisatie voor Wetenschappelijk Onderzoek
- Leids Universitair Medisch Centrum
- Universitair Medisch Centrum Groningen
- Radboud Universitair Medisch Centrum
- Nederlandse Vereniging voor Trombose en Hemostase
- Swedish Orphan Biovitrum
- Stichting Haemophilia
- Erasmus Universitair Medisch Centrum Rotterdam