METALLOPROTEASE ADAMTS-13

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...

ADAMTS-13 gene expression in antiphospholipid syndrome Antifosfolipid sendromunda ADAMTS-13 gen ekspresyonu

Veysel Sabri Hançer1, Reyhan Diz Küçükkaya2, Ayşegül Topal-Sarıkaya3 1Department of Medical Biology and Genetics, Faculty of Medicine, İstanbul Bilim University, İstanbul, Turkey 2Department of Internal Medicine, Division of Hematology, Faculty of Medicine, İstanbul Bilim University, İstanbul, Turkey 3Department of Molecular Biology and Genetics, Faculty of Science, İstanbul University, İstanbu...

Recombinant ADAMTS 13 in thrombotic thrombocytopenic purpura

Recombinant ADAMTS 13 Attenuates Brain Injury After Intracerebral Hemorrhage.

BACKGROUND AND PURPOSE Inflammatory responses and blood-brain barrier (BBB) dysfunction play important roles in brain injury after intracerebral hemorrhage (ICH). The metalloprotease ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type I motif, member 13) was shown to limit inflammatory responses through its proteolytic effects on von Willebrand factor. In the present study, we...

Conformational quiescence of ADAMTS‐13 prevents proteolytic promiscuity

Essentials Recently, ADAMTS-13 has been shown to undergo substrate induced conformation activation. Conformational quiescence of ADAMTS-13 may serve to prevent off-target proteolysis in plasma. Conformationally active ADAMTS-13 variants are capable of proteolysing the Aα chain of fibrinogen. This should be considered as ADAMTS-13 variants are developed as potential therapeutic agents. Click to ...