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ADAMTS13 assays and ADAMTS13-deficient mice
Current Opinion in Hematology, 2007Thrombotic thrombocytopenic purpura can be induced by acquired or congenital deficiency of the plasma von Willebrand factor-cleaving protease, ADAMTS13. Measurement of ADAMTS13 activity is important for the diagnosis and treatment of microangiopathies including thrombotic thrombocytopenic purpura.
Toshiyuki, Miyata +4 more
openaire +2 more sources
Blood, 2005
It has now been 3 years since the von Willebrand factor (VWF)–cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (adisintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 ADAMTS13 mutations resulting in familial TTP have been reported.
Gallia G, Levy +2 more
openaire +2 more sources
It has now been 3 years since the von Willebrand factor (VWF)–cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (adisintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 ADAMTS13 mutations resulting in familial TTP have been reported.
Gallia G, Levy +2 more
openaire +2 more sources
Current Opinion in Hematology, 2002
Thrombotic thrombocytopenic purpura (TTP) has been a mysterious and deadly disease that often could be treated effectively by plasma exchange, but without real understanding of the underlying pathophysiology. Recent advances now suggest that deficiency of a specific von Willebrand factor (VWF) cleaving protease promotes tissue injury in TTP.
Xinglong, Zheng +2 more
openaire +2 more sources
Thrombotic thrombocytopenic purpura (TTP) has been a mysterious and deadly disease that often could be treated effectively by plasma exchange, but without real understanding of the underlying pathophysiology. Recent advances now suggest that deficiency of a specific von Willebrand factor (VWF) cleaving protease promotes tissue injury in TTP.
Xinglong, Zheng +2 more
openaire +2 more sources
Pharmacogenomics, 2011
The multidomain metalloprotease ADAMTS13 limits thrombus formation via the cleavage of large multimeric forms of von Willebrand factor. Deficiency of functional ADAMTS13 is associated with a number of disease pathologies including thrombotic thrombocytopenic purpura, cardiovascular disease and inflammation.
Sandra Chang, Tseng +1 more
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The multidomain metalloprotease ADAMTS13 limits thrombus formation via the cleavage of large multimeric forms of von Willebrand factor. Deficiency of functional ADAMTS13 is associated with a number of disease pathologies including thrombotic thrombocytopenic purpura, cardiovascular disease and inflammation.
Sandra Chang, Tseng +1 more
openaire +2 more sources
ADAMTS13 Activity: Screening Test Protocol
2023Accurate estimation of ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13) activity level is necessary for diagnosis and management of thrombotic microangiopathies (TMA). In particular, it permits distinction between thrombotic thrombocytopenic purpura (TTP) and other TMAs, prompting disorder appropriate ...
Gary W, Moore +2 more
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Blood, 2009
In mice, a long form and a short form of the VWF-cleaving protease ADAMTS13 have been identified, the latter lacking the 4 distal carboxyl-terminal domains. While these are not strictly required for regulating normal size distribution of VWF multimers, in this issue of Blood, Banno and colleagues reveal the role of these domains in down-regulating ...
Karen, Vanhoorelbeke +2 more
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In mice, a long form and a short form of the VWF-cleaving protease ADAMTS13 have been identified, the latter lacking the 4 distal carboxyl-terminal domains. While these are not strictly required for regulating normal size distribution of VWF multimers, in this issue of Blood, Banno and colleagues reveal the role of these domains in down-regulating ...
Karen, Vanhoorelbeke +2 more
openaire +2 more sources
Transcriptional regulation of ADAMTS13
Thrombosis and Haemostasis, 2005SummaryThe metalloproteinase ADAMTS13 cleaves VWF multimers instantaneously when they are released from endothelial cells. Absent or manifestly diminished proteolytic activity of ADAMTS13 results in the appearance and accumulation of ultralarge VWF multimers (ULVWFM) in plasma, characterised by the manifestation of Thrombotic Thrombocytopenic Purpura ...
Claus, Ralf A. +7 more
openaire +3 more sources