ADAMTS-13 and von Willebrand factor: a dynamic duo

ADAMTS‐13 and von Willebrand factor: a dynamic duo

von Willebrand factor (VWF) is a key player in hemostasis, acting as a carrier for factor VIII and capturing platelets at sites of vascular damage. To capture platelets, it must undergo conformational changes, both within its A1 domain and at the macromolecular level through A2 domain unfolding. Its size and this function are regulated by the metalloproteinase ADAMTS-13. Recently, it has been s...

1 Title : N - Glycans of ADAMTS 13 Modulate its Secretion and von Willebrand Factor Cleaving Activity

The von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP).

Thrombotic thrombocytopenic purpura (TTP) is a life threatening disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia as a result of microvascular platelet clumping often accompanied by ischemic organ dysfunctions such as neurological abnormalities or renal insufficiency, and fever. Until the sixties of the 20th century TTP remained an almost universally fatal disorde...

Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease

Changes in plasma von Willebrand factor concentration (VWF:Ag) and ADAMTS-13 activity (the metalloprotease that cleaves VWF physiologically) have been reported in several cardiovascular disorders with prognostic implications. We therefore determined the level of these proteins in the plasma of children with cyanotic congenital heart disease (CCHD) undergoing surgical treatment. Forty-eight chil...

Severe Dengue Is Associated with Consumption of von Willebrand Factor and Its Cleaving Enzyme ADAMTS-13

BACKGROUND Thrombocytopenia, bleeding and plasma leakage are cardinal features of severe dengue. Endothelial cell activation with exocytosis of Weibel-Palade bodies (WPBs) may play an etiological role in this condition. METHODS AND PRINCIPAL FINDINGS In a cohort of 73 Indonesian children with dengue hemorrhagic fever (DHF), of which 30 with dengue shock syndrome (DSS), we measured plasma leve...