Reactive oxygen species (ROS) and mitochondria play a pivotal role in regulating platelet
functions. Platelet activation determines a drastic change in redox balance and in platelet …

Hemostasis and pathological thrombus formation are dynamic processes that require
multiple adhesive receptor-ligand interactions, with blood platelets at the heart of such …

Filaminopathies A caused by mutations in the X-linked FLNA gene are responsible for a
wide spectrum of rare diseases including 2 main phenotypes, the X-linked dominant form of …

Over the last 100 years the role of platelets in hemostatic events and their production by
megakaryocytes have gradually been defined. Progressively, thrombocytopenia was …

Inherited diseases of the megakaryocyte lineage give rise to bleeding when platelets fail to
fulfill their hemostatic function upon vessel injury. Platelet defects extend from the absence …

Abstract von Willebrand factor (vWF) was first identified as an adhesive glycoprotein
involved in hemostasis by Zimmermann in 1971. Since then, vWF has been shown to play a …

Genetic defects of platelets constitute rare diseases that include bleeding syndromes of
autosomal dominant, recessive or X‐linked inheritance. They affect platelet production …

Abstract The glycoprotein (GP) Ib‐IX receptor complex plays a critical role in platelet
physiology and pathology. Its interaction with von Willebrand factor (VWF) on the …

Abstract von Willebrand disease is a common inherited bleeding disorder characterized by
excessive mucocutaneous bleeding. Characteristic bleeding symptoms include epistaxis …

Genetic defects of platelet function give rise to mucocutaneous bleeding of varying severity
because platelets fail to fulfil their haemostatic role after vessel injury. Abnormalities of …