Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Severe deficiency of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 can lead to thrombotic thrombocytopenic purpura (TTP), a disease associated with the widespread formation of platelet-rich thrombi in many organs.
|
14976043 |
2004 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
LHGDN |
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 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In patients with thrombotic thrombocytopenic purpura (TTP), unusually large multimers of von Willebrand factor (VWF) circulate in the plasma.
|
14727257 |
2004 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Thrombotic thrombocytopenic purpura (TTP) is a severe disease associated with unusually large, hemostatically hyperactive von Willebrand factor (VWF) and severe deficiency in ADAMTS-13, the protease responsible for the proteolytic degradation of VWF in plasma.
|
14727256 |
2004 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Deficiency of ADAMTS13, due to autoimmune inhibitors of the protease or genetic mutation in the ADAMTS13 gene, results in a propensity to the development of von Willebrand factor-platelet aggregation and intravascular thrombosis characteristic of TTP.
|
15497097 |
2004 |
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 |
The deficiency of ADAMTS13 leaves unchecked the hyperadhesive vWF unfolded under high shear stress in the microvessels, resulting in the formation of platelet thrombi, which in turn causes TTP.
|
16388413 |
2005 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Building on these studies, continued investigation of ADAMTS13 and VWF holds considerable promise for advancing the understanding of TTP pathogenesis and should lead to improved diagnosis and treatment for this important hematologic disease.
|
15774620 |
2005 |
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 |
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 |
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 |
In fact, while the presence in plasma of unusually large VWF multimers due to a congenital or acquired deficiency of a VWF-cleaving metalloprotease has been implicated in the pathogenesis of thrombotic thrombocytopenic purpura (TTP), high plasma levels of VWF have been associated with a slightly increased risk of arterial thrombosis.
|
16568319 |
2006 |
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 |
Together, these findings shed new light on the potential roles played by ADAMTS13 and VWF in TTP, endotoxemia, and normal hemostasis.
|
18083848 |
2008 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Thrombotic thrombocytopenic purpura (TTP) is often associated with acquired or congenital deficiency of the von Willebrand factor-cleaving metalloprotease, ADMATS13 (Lammle B et al., J Thromb Haemost 2005;3:1663-1675; Schneppenheim et al., Blood 2003;101:1845-1850).
|
18756543 |
2008 |
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 |
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 |
GeneticVariation
|
disease |
BEFREE |
Recent studies have demonstrated that thrombotic thrombocytopenic purpura (TTP), a serious thrombotic disorder affecting the arterioles and capillaries of multiple organs, is caused by a profound deficiency in the von Willebrand factor cleaving metalloprotease, ADAMTS13.
|
19180123 |
2009 |
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 |
In the past 10 years the molecular bases of aHUS and TTP have been discovered that mostly lead to uncontrolled activation of the complement system in aHUS and to abnormal von Willebrand factor processing in TTP.
|
20807612 |
2010 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Our results demonstrate that residues within Y658-Y665 of the ADAMTS13 spacer domain that are targeted by autoantibodies in TTP directly interact with a complementary exosite (E1660-R1668) within the VWF A2 domain.
|
20032502 |
2010 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Recent studies demonstrate that Stx also induces von Willebrand factor (VWF) secretion by human endothelial cells and causes thrombotic thrombocytopenic purpura, a disease with similarities to D+HUS, in Adamts13(-/-) mice.
|
20644116 |
2010 |
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 endothelial cell damage is considered the reason for complement and platelet activation leading to thrombus formation.
|
20539230 |
2010 |
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 |
Biomarker
|
disease |
BEFREE |
This review discusses potentials of using recombinant ADAMTS-13 and VWF-blocking agents as therapeutics for TTP and other acquired ADAMTS-13 deficiencies.
|
22022775 |
2012 |