Molecular mechanisms underlying the platelet procoagulant response: back to basics

N. J. A. Mattheij

Research output: ThesisDoctoral ThesisInternal

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Differently sized platelets are present during activated coagulation, such as aggregated cells, activated clotting platelets and platelet-fibrinogen interactions. The stability of a blood clot is determined by the generation of a coagulation-active surface and the inactivation of integrin receptors. Platelets increase their surface area through balloon formation to improve the binding capacity of coagulation factors.

Patients with Scott syndrome, a rare congenital bleeding disorder, and a defect in the protein anoctamine-6 have platelets with significantly reduced blood coagulation. Using in-vitro and ex-vivo experiments and genetically modified mice, we found a direct link between the presence of anoctamine-6 and the development of coagulation-active platelets and haemorrhage. The absence of anoctamine-6 results in several changes to the protein levels of Scott platelets, which may explain this complex phenotype.
Original languageEnglish
QualificationDoctor of Philosophy
Awarding Institution
  • Maastricht University
  • Heemskerk, Johan, Supervisor
  • Cosemans, Judith, Co-Supervisor
Award date29 Jan 2016
Place of PublicationMaastricht
Print ISBNs9789064649615
Publication statusPublished - 1 Jan 2016


  • platelets
  • coagulation
  • Scott syndrome

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