Mechanisms of autoantibody-induced pathology

RJ Ludwig, K Vanhoorelbeke, F Leypoldt… - Frontiers in …, 2017 - frontiersin.org
Autoantibodies are frequently observed in healthy individuals. In a minority of these
individuals, they lead to manifestation of autoimmune diseases, such as rheumatoid arthritis …

Thrombotic thrombocytopenic purpura

JA Kremer Hovinga, P Coppo, B Lämmle… - Nature reviews Disease …, 2017 - nature.com
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is
characterized by the concomitant occurrence of often severe thrombocytopenia …

Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor

JTB Crawley, R de Groot, Y Xiang… - Blood, The Journal …, 2011 - ashpublications.org
Abstract von Willebrand factor (VWF) is a large adhesive glycoprotein with established
functions in hemostasis. It serves as a carrier for factor VIII and acts as a vascular damage …

ADAMTS proteases in cardiovascular physiology and disease

S Santamaria, R de Groot - Open biology, 2020 - royalsocietypublishing.org
The a disintegrin-like and metalloproteinase with thrombospondin motif (ADAMTS) family
comprises 19 proteases that regulate the structure and function of extracellular proteins in …

ADAM and ADAMTS family proteins and snake venom metalloproteinases: a structural overview

S Takeda - Toxins, 2016 - mdpi.com
A disintegrin and metalloproteinase (ADAM) family proteins constitute a major class of
membrane-anchored multidomain proteinases that are responsible for the shedding of cell …

Management of thrombotic thrombocytopenic purpura: current perspectives

P Blombery, M Scully - Journal of blood medicine, 2014 - Taylor & Francis
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic
microangiopathy which causes significant morbidity and mortality unless promptly …

Conformational activation of ADAMTS13

K South, BM Luken, JTB Crawley… - Proceedings of the …, 2014 - National Acad Sciences
A disintegrin and metalloprotease with thrombospondin motifs 13 (ADAMTS13) is a
metalloprotease that regulates von Willebrand factor (VWF) function. ADAMTS13-mediated …

Structure–function and regulation of ADAMTS‐13 protease

XL Zheng - Journal of Thrombosis and Haemostasis, 2013 - Wiley Online Library
Summary ADAMTS‐13, a plasma reprolysin‐like metalloprotease, cleaves von W illebrand
factor (VWF). Severe deficiency of plasma ADAMTS‐13 activity results in thrombotic …

Pathogenicity of anti-ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura

MR Thomas, R de Groot, MA Scully, JTB Crawley - EBioMedicine, 2015 - thelancet.com
Background Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune
disease in which anti-ADAMTS13 autoantibodies cause severe enzyme deficiency …

Crystal structure and substrate-induced activation of ADAMTS13

A Petri, HJ Kim, Y Xu, R de Groot, C Li… - Nature …, 2019 - nature.com
Platelet recruitment to sites of blood vessel damage is highly dependent upon von
Willebrand factor (VWF). VWF platelet-tethering function is proteolytically regulated by the …