|
Thrombotic Microangiopathies
|
0.100 |
GeneticVariation
|
group |
BEFREE |
In the acquired form, autoantibodies against ADAMTS13 inhibit cleaving of von Willebrand factor (vWF) multimers, thereby promoting their interaction with thrombocytes, causing TMA and MAHA.
|
31177334 |
2019 |
|
Thrombotic Microangiopathies
|
0.100 |
AlteredExpression
|
group |
BEFREE |
The importance of unravelling these recent findings for our understanding of TMA pathology becomes even more evident considering that glomerular ECs express VWF in a heterogeneous pattern with an overall decreased expression level, thus potentially leaving the glomerular ECs vulnerable to complement-mediated injury.
|
28748411 |
2018 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
In pathologic states such as thrombotic thrombocytopenic purpura (TTP) and other thrombotic microangiopathies (TMAs), VWF can bind to the endothelium and form large multimers.
|
30208220 |
2018 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
A deficiency in ADAMTS-13 leads to higher concentrations of ultralarge VWF multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura, a thrombotic microangiopathy characterized by thrombocytopenia, non-immune hemolysis, and organ dysfunction.
|
29337416 |
2018 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
In sepsis, the severity-dependent decrease of von Willebrand factor (VWF)-inactivating protease, a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13), results in platelet aggregation and consumption, leading to sepsis-associated thrombotic microangiopathy (TMA) and organ failure.
|
25860876 |
2015 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
Upshaw-Schulman syndrome (USS) is due to severe congenital deficiency of von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 domains, nr 13) activity resulting in the presence of unusually large forms of VWF in the circulation, causing intravascular platelet clumping and thrombotic microangiopathy.
|
24033710 |
2014 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
Plasmin cleavage of von Willebrand factor as an emergency bypass for ADAMTS13 deficiency in thrombotic microangiopathy.
|
24449821 |
2014 |
|
Thrombotic Microangiopathies
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway.
|
24967890 |
2014 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
The next generation of drugs focuses on using recombinant ADAMTS13 and molecules that block the interaction of VWF and platelets to prevent thrombotic microangiopathy.
|
23420593 |
2013 |
|
Thrombotic Microangiopathies
|
0.100 |
GeneticVariation
|
group |
BEFREE |
This proteolytic cleavage is essential to reduce the size of ultralarge VWF polymers, which, when exposed to high shear stress in the microcirculation, are prone to form platelets clumps, which cause severe syndromes called thrombotic microangiopathies (TMAs).
|
21238935 |
2011 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice.
|
20644116 |
2010 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
These advances illustrate that dysregulation of VWF homeostasis or complement activation owing to genetic or autoimmune mechanisms may lead to the syndrome of thrombotic microangiopathy.
|
16760911 |
2006 |
|
Thrombotic Microangiopathies
|
0.100 |
Biomarker
|
group |
BEFREE |
Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies.
|
15662617 |
2005 |