Purpura, Thrombotic Thrombocytopenic
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The regulation of VWF multimer size is essential in preventing spontaneous microvascular platelet clumping, a central pathophysiologic finding in thrombotic thrombocytopenic purpura (TTP).
|
23233642 |
2012 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Thrombotic thrombocytopenic purpura (TTP) is a disorder with characteristic von Willebrand factor (VWF)-rich microthrombi affecting the arterioles and capillaries of multiple organs.
|
20058209 |
2010 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Histone-induced thrombotic thrombocytopenic purpura in adamts13-/- zebrafish depends on von Willebrand factor.
|
31753928 |
2019 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
The presence of unusually large multimers of von Willebrand factor (VWF) is thought to be a major pathogenic factor for thrombotic thrombocytopenic purpura (TTP).
|
15662617 |
2005 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
We conducted this study to examine if vWF is involved in the increased platelet retention in TTP.
|
9423794 |
1997 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Recent studies have found autoimmune inhibitors or genetic mutations of a von Willebrand factor (VWF) cleaving metalloprotease ADAMTS13 in patients with TTP.
|
16760911 |
2006 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Despite a clinical diagnosis of TTP, the patient had normal ADAMTS-13 levels and increased VWF antigen levels with ultra-large von Willebrand factor multimers.
|
26613809 |
2016 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Severely deficient activity of the von Willebrand Factor (VWF) cleaving metalloprotease, ADAMTS13, is associated with thrombotic thrombocytopenic purpura (TTP).
|
16807643 |
2006 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Force-regulated cleavage of A2 domain of von Willebrand factor (vWF) by ADAMTS13 is a key event in preventing thrombotic thrombocytopenic purpura (TTP).
|
29636514 |
2018 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Thrombotic thrombocytopenic purpura (TTP) is associated with congenital and acquired deficiency of ADAMTS-13, a metalloprotease that cleaves von Willebrand factor (VWF) and reduces its adhesive activity.
|
16796708 |
2006 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
The identification of antibodies against a plasma metalloproteinase responsible for cleaving ultralarge von Willebrand factor multimers in thrombotic thrombocytopenic purpura has been followed by the elucidation of the identity of the proteinase.
|
12105394 |
2002 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
<i>N</i>-acetylcysteine (NAC), an FDA-approved anti-mucolytic agent, is a possible new treatment strategy for TTP, as it was demonstrated to reduce disulfide bonds in VWF, thereby decreasing VWF multimers size and hence their prothrombotic potential.
|
28011677 |
2017 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Severe deficiency of von Willebrand factor (VWF)-cleaving protease (ADAMTS-13) activity (<5% of normal) is a specific finding for acute idiopathic thrombotic thrombocytopenic purpura (TTP), a disorder that presents as thrombocytopenia, microangiopathic hemolytic anemia, and often organ dysfunction such as neurological disturbances or renal failure, and fever.
|
14727262 |
2004 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Acquired thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease in which ultralarge von Willebrand factor (UL-VWF) multimers accumulate as a result of autoantibody inhibition of the VWF protease, ADAMTS13.
|
27040023 |
2016 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Recent studies demonstrate that assay of ADAMTS13, a circulating zinc metalloprotease that cleaves von Willebrand factor (VWF) at the Y1605-M1606 bond, is an important tool in the diagnosis of thrombotic thrombocytopenic purpura (TTP).
|
15045144 |
2004 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The congenital or acquired deficiency of the von Willebrand factor (VWF) cleaving protease, ADAMTS-13 has been specifically associated with a diagnosis of thrombotic thrombocytopenic purpura (TTP), a microangiopathy characterized by the formation of occlusive platelet thrombi.
|
15521921 |
2004 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Hereditary thrombotic thrombocytopenic purpura is caused by mutations in a disintegrin and metalloprotease with thrombospondin motifs (ADAMTS13) resulting in defective processing of von Willebrand factor (VWF) that causes intravascular platelet aggregation culminating in thrombocytopenia with shistocytic anemia.
|
19786614 |
2009 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Severe deficiency of ADAMTS13, a von Willebrand factor (VWF)-cleaving metalloprotease, causes thrombotic thrombocytopenic purpura.
|
17426255 |
2007 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
In TTP thrombi formation occurs subsequently to the release of multimers of von Willebrand Factor (vWF) and in HUS (both typical and atypical) to endothelial cell damage (via toxins or complement dysregulation).
|
19640589 |
2009 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
As the anchored VWF chains grow, they provide a greater surface area to bind circulating platelets (PLTs), generating unique thrombi that characterize TTP.
|
30208220 |
2018 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
ADAMTS13 and von Willebrand factor (VWF) are closely related to the onset of TTP.
|
26759371 |
2017 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
In some variants of type 2A vWF, the cleavage site in the vWF subunit is more susceptible to proteolytic degradation than in normal vWF, whereas in patients with TTP or HUS the protease activity may be suppressed. vWF-degrading protease plays an important role in pathogenesis of congenital or acquired disorders of hemostasis and thrombosis.
|
8767102 |
1996 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
We investigated whether VWF-HMWM level at acute phase of TTP could be a predictive factor for morbidity.
|
31330376 |
2019 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Finally, (4) studies of patients with primary disorders of excess or deficiency of function in the vWF axis (e.g. thrombotic thrombocytopenic purpura and von Willebrand disease, respectively) which demonstrate the crucial role of vWF in atherothrombosis.
|
29847840 |
2018 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Failure to degrade "unusually large" (UL) von Willebrand factor (VWF) multimers as they are secreted from endothelial cells probably causes most cases of familial TTP, acquired idiopathic TTP, thienopyridine-related TTP, and pregnancy-associated TTP.
|
14727254 |
2004 |