Publications by authors named "M Boon-Spijker"
Article Synopsis
- von Willebrand disease (VWD) is a bleeding disorder that happens when a protein called von Willebrand factor (VWF) is missing or doesn’t work right in the body.
- The study looked at how changes in the VWF gene affect the different types of VWF proteins found in the blood of patients, using advanced testing methods.
- The researchers found that there are many different versions of VWF proteins in patients, which can help understand how this disease works and how to better diagnose and treat it.
Background: Biomonitoring may provide important insights into the impact of a whole blood donation for individual blood donors.
Study Design And Methods: Here, we used unbiased mass spectrometry (MS)-based proteomics to assess longitudinal changes in the global plasma proteome, after a single blood donation for new and regular donors. Subsequently, we compared plasma proteomes of 76 male and female whole blood donors, that were grouped based on their ferritin and hemoglobin (Hb) levels.
Background: Activated factor IX (FIXa) is an inefficient enzyme that needs activated factor VIII (FVIII) for full activity. Recently, we identified a network of FVIII-driven changes in FIXa employing hydrogen-deuterium eXchange mass spectrometry (HDX-MS). Some changes also occurred in active-site inhibited FIXa, but others were not cofactor-driven, in particular those within the 220-loop (in chymotrypsin numbering).
View Article and Find Full Text PDFHydrogen-deuterium exchange mass spectrometry (HDX-MS) was employed to gain insight into the changes in factor VIII (FVIII) that occur upon its activation and assembly with activated factor IX (FIXa) on phospholipid membranes. HDX-MS analysis of thrombin-activated FVIII (FVIIIa) revealed a marked increase in deuterium incorporation of amino acid residues along the A1-A2 and A2-A3 interface. Rapid dissociation of the A2 domain from FVIIIa can explain this observation.
View Article and Find Full Text PDFThe assembly of the enzyme-activated factor IX (FIXa) with its cofactor, activated factor VIII (FVIIIa) is a crucial event in the coagulation cascade. The absence or dysfunction of either enzyme or cofactor severely compromises hemostasis and causes hemophilia. FIXa is a notoriously inefficient enzyme that needs FVIIIa to drive its hemostatic potential, by a mechanism that has remained largely elusive to date.
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