ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.
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Pathophysiology of thrombotic thrombocytopenic purpuraPathogenesis of thrombotic microangiopathiesProteolytic processing of von Willebrand factor by adamts13 and leukocyte proteasesThe von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP)Identification of strain-specific variants of mouse Adamts13 gene encoding von Willebrand factor-cleaving proteaseMeasurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods.Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).Thrombotic thrombocytopenic purpura: yesterday, today, tomorrow.FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay.Cloning, expression and functional characterization of the full-length murine ADAMTS13.The course of ADAMTS-13 activity and inhibitor titre in the treatment of thrombotic thrombocytopenic purpura with plasma exchange and vincristine.Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: a novel therapeutic strategy?Molecular biology of ADAMTS13 and diagnostic utility of ADAMTS13 proteolytic activity and inhibitor assays.Systemic antithrombotic effects of ADAMTS13.ADAMTS13 and microvascular thrombosisThe kidney in thrombotic thrombocytopenic purpura.Targeting von Willebrand factor and platelet glycoprotein Ib receptor.Recombinant ADAMTS13 normalizes von Willebrand factor-cleaving activity in plasma of acquired TTP patients by overriding inhibitory antibodies.Investigational drugs in thrombotic thrombocytopenic purpura.Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan.ADAMTS-13 in the Diagnosis and Management of Thrombotic Microangiopathies.How I treat refractory thrombotic thrombocytopenic purpura.Why Do We Need ADAMTS13?The cooperative activity between the carboxyl-terminal TSP1 repeats and the CUB domains of ADAMTS13 is crucial for recognition of von Willebrand factor under flow.SNPs in ADAMTS13.Biological functions of fucose in mammals.Apical sorting of ADAMTS13 in vascular endothelial cells and Madin-Darby canine kidney cells depends on the CUB domains and their association with lipid rafts.Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13.Thrombotic thrombocytopenic purpuraThrombotic thrombocytopenic purpuraThrombotic microangiopathies
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P2860
ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.
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2003 nî lūn-bûn
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2003 թուականի Մարտին հրատարակուած գիտական յօդուած
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2003 թվականի մարտին հրատարակված գիտական հոդված
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2003年の論文
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2003年論文
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2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
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2003年論文
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2003年论文
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name
ADAMTS13 gene defects in two b ...... by recombinant human ADAMTS13.
@ast
ADAMTS13 gene defects in two b ...... by recombinant human ADAMTS13.
@en
type
label
ADAMTS13 gene defects in two b ...... by recombinant human ADAMTS13.
@ast
ADAMTS13 gene defects in two b ...... by recombinant human ADAMTS13.
@en
prefLabel
ADAMTS13 gene defects in two b ...... by recombinant human ADAMTS13.
@ast
ADAMTS13 gene defects in two b ...... by recombinant human ADAMTS13.
@en
P2093
P2860
P1476
ADAMTS13 gene defects in two b ...... by recombinant human ADAMTS13.
@en
P2093
Friedrich Scheiflinger
Gerhard Antoine
Jan-Dirk Studt
Klaus Zimmermann
Monika Grillowitzer
P2860
P304
P356
10.1046/J.1365-2141.2003.04183.X
P407
P577
2003-03-01T00:00:00Z