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 |
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 |
Biomarker
|
disease |
BEFREE |
Our data suggest that the unusually large vWF multimers found in TTP patients may be caused by deficient vWF-cleaving protease activity.
|
9129011 |
1997 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Plasma from patients with classic or BMT-associated TTP was incubated with recombinant vWF R834Q, a vWF mutant with enhanced sensitivity to the protease.
|
10339486 |
1999 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP)
|
10595623 |
1999 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Increased fragmentation of von Willebrand factor, due to abnormal cleavage of the subunit, parallels disease activity in recurrent hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and discloses predisposition in families. The Italian Registry of Familial and Recurrent HUS/TTP.
|
10397728 |
1999 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Proteolysis of the blood-clotting protein von Willebrand factor (VWF) observed in normal plasma is decreased in TTP patients.
|
11586351 |
2001 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We report the results of 10 years of prophylactic fresh-frozen plasma (FFP) infusion therapy in a 14-year-old girl with chronic relapsing thrombotic thrombocytopenic purpura (TTP), in whom a severe congenital von Willebrand factor (VWF)-cleaving protease deficiency has been documented.
|
11380451 |
2001 |
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 |
Deficient von Willebrand factor (VWF) degradation has been associated with thrombotic thrombocytopenic purpura (TTP).
|
12393399 |
2002 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Recent studies on the relationship between UL-vWFMs and vWF-CPase, together with its autoantibody (inhibitor) have brought about a clear discrimination between thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
|
11843286 |
2002 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Recent advances now suggest that deficiency of a specific von Willebrand factor (VWF) cleaving protease promotes tissue injury in TTP.
|
12172456 |
2002 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
These findings support the view that vWF proteolysis is critical in regulating vWF-platelet interaction and set the stage for improving the diagnosis and treatment of thrombotic thrombocytopenic purpura.
|
12395148 |
2002 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
This observation led to the recognition that UL vWF multimers precipitate the thrombotic occlusion of arterioles, a feature that characterizes TTP.
|
12656756 |
2003 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Thrombotic thrombocytopenic purpura (TTP) is caused by the persistence of the highly reactive high-molecular-weight multimers of von Willebrand factor (VWF) due to deficiency of the specific VWF-cleaving protease (VWF-CP) ADAMTS13, resulting in microangiopathic disease.
|
12393505 |
2003 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
The metalloprotease ADAMTS-13 cleaves von Willebrand factor (VWF), and is absent or severely reduced in the plasma of patients with thrombotic thrombocytopenia purpura (TTP).
|
14652658 |
2003 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Deficiency of von Willebrand factor (VWF) cleaving protease ADAMTS13 is associated with the development of thrombotic thrombocytopenic purpura (TTP).
|
12576319 |
2003 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Deficiency of a recently identified novel metalloprotease, the von Willebrand factor (vWF)-cleaving protease, originating from mutations in the ADAMTS13 gene plays a major role in the development of TTP.
|
12753286 |
2003 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
This article reviews the role of this cleavage in regulating vWF-platelet interaction and proposes a scheme for understanding how a deficiency of ADAMTS13 results in the development of microthrombi in patients with thrombotic thrombocytopenic purpura.
|
12615692 |
2003 |
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 |
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 |
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 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Deficiency of von Willebrand factor (VWF) cleaving protease ADAMTS13 has been demonstrated to be the proximate cause of a subset of thrombotic microangiopathic haemolytic anaemias (MAHA) typical for thrombotic thrombocytopenic purpura (TTP).
|
15009067 |
2004 |
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Severe deficiency of the von Willebrand Factor (VWF)-cleaving proteinase, ADAMTS13, is associated with the development of thrombotic thrombocytopenic purpura (TTP).
|
14512317 |
2004 |