ADAMTS13

The protease A13 has a key function in regulating von Willebrand factor (VWF) activity in normal hemostasis and when dysregulated in disorders such as inherited and acquired A13 deficiency, serious clinical conditions result such as thrombotic thrombocytopenic purpura. ADAMTS-13: Biology and Disease summarizes the current status of A13 research both basic and clinical in one volume for interested scientists and clinicians.§§This essential volume starts by covering basic science topics including the biochemistry and structure-function relationships of A13, as well as its normal function in hemostasis (cleavage of VWF). Subsequently, emerging research themes for A13 are also covered, including its potential role in angiogenesis and other aspects of cell biology. Clinical topics include laboratory assays for A13, inherited A13 deficiency and acquired A13 deficiency. A chapter on related thrombotic microangiopathic (TMA) disorders is included so that readers can appreciate differences between TMAs associated with A13 deficiency from those not associated with A13 deficiency. The latter clinical chapters summarize treatment aspects, both standard and novel. A final chapter reviews the preliminary information on emerging aspects of A13; for example, the status of recombinant A13 products and their potential utility. This will be an important addition to the libraries of basic scientists or physician investigators with an interest in hemostasis, vascular biology, biochemistry, metalloproteases; clinical hematologists adult and pediatric and transfusion medicine physicians.§