GC1126A, a novel ADAMTS13 mutein, evades autoantibodies in immune-mediated thrombotic thrombocytopenic purpura [PDF]
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening blood disorder characterized by the formation of blood clots in small blood vessels. It is caused by antibodies targeting the A disintegrin and metalloprotease with
Heechun Kwak+13 more
doaj +2 more sources
ADAMTS13 Deficiency Worsens Colitis and Exogenous ADAMTS13 Administration Decreases Colitis Severity in Mice [PDF]
Background Inflammatory bowel disease (IBD) affects 1.6 million people in the United States. IBD is associated with an increased risk of thrombosis, which rises with disease activity. The pathogenesis of IBD and its increased thrombotic risk is not completely understood. Ultra large von Willebrand factor (ULVWF) multimers are secreted from
Kimberly Martinod+14 more
openaire +6 more sources
A Rare But Fatal Toxicity: Immune Checkpoint Inhibitor–Related Acquired Thrombotic Thrombocytopenic Purpura [PDF]
Thrombotic thrombocytopenic purpura (TTP) is characterized by thrombotic microangiopathy resulting from decreased activation of the von Willebrand factor–cleaving protease (ADAMTS13).
Aysun Senturk Yikilmaz+1 more
doaj +2 more sources
Dexmedetomidine Reduces Chronic Stress–Related Thrombosis in a Mouse FeCl3 Model [PDF]
Schematic summary of the mechanism by which dexmedetomidine prevents chronic stress–related carotid artery thrombosis in a mouse FeCl3 model. These findings present evidence and a potential mechanistic explanation of the Dex‐mediated amelioration of arterial thrombosis associated with the reduction of oxidative stress, inflammation, and apoptosis, and ...
Huazhen Wang+11 more
wiley +2 more sources
A Descriptive 5‐Year Analysis of the Demographics and Therapies for Patients With Immune Thrombotic Thrombocytopenic Purpura in the USA: A Multicenter Study of 390 Disease Episodes From 2017 to 2021 [PDF]
ABSTRACT Immune thrombotic thrombocytopenic purpura (iTTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular occlusion secondary to acquired ADAMTS13 deficiency. Contemporary data regarding iTTP treatment practices in the US, including the use of caplacizumab, are lacking.
Jeremy W. Jacobs+34 more
wiley +2 more sources
Background: Plasma levels of von Willebrand factor (VWF) are significantly elevated in patients with coronavirus disease 2019 (COVID-19). However, dynamic changes and prognostic value of this biomarker in hospitalized patients with COVID-19 have not been
Quan Zhang+6 more
semanticscholar +1 more source
Impaired exercise capacity in post–COVID-19 syndrome: the role of VWF-ADAMTS13 axis
Post-COVID syndrome (PCS) or Long-COVID is an increasingly recognised complication of acute SARS-CoV-2 infection, characterised by persistent fatigue, reduced exercise tolerance chest pain, shortness of breath and cognitive slowing.
Nithya Prasannan+11 more
semanticscholar +1 more source
The Intriguing Connections between von Willebrand Factor, ADAMTS13 and Cancer
von Willebrand factor (VWF) is a complex and large protein that is cleaved by ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13), and together they serve important roles in normal hemostasis.
Chanukya K Colonne+2 more
semanticscholar +1 more source
Recombinant ADAMTS13 for Hereditary Thrombotic Thrombocytopenic Purpura.
A 27-year-old patient with a history of severe obstetrical complications and arterial thrombosis received a diagnosis of hereditary thrombotic thrombocytopenic purpura (TTP) due to severe ADAMTS13 deficiency when she presented with an acute episode in ...
L. Asmis+9 more
semanticscholar +1 more source
Aging and obesity independently contribute toward an endothelial dysfunction that results in an imbalanced VWF to ADAMTS13 ratio. In addition, plasma thrombin and plasmin generation are elevated and reduced, respectively, with increasing age and also ...
Kiruphagaran Thangaraju+20 more
semanticscholar +1 more source