Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Sign Up to like & getrecommendations! Published in 2018 at "Thrombosis and haemostasis"
DOI: 10.1055/s-0038-1669459
Abstract: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency, the presence of anti-ADAMTS13 autoantibodies and an open ADAMTS13 conformation with a cryptic… read more here.
Keywords: adamts13; immune mediated; anti adamts13; adamts13 autoantibodies ... See more keywords
Inverse Regulation of Confluence-Dependent ADAMTS13 and von Willebrand Factor Expression in Human Endothelial Cells.
Sign Up to like & getrecommendations! Published in 2021 at "Thrombosis and haemostasis"
DOI: 10.1055/s-0041-1733800
Abstract: ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) is a zinc-containing metalloprotease also known as von Willebrand factor (vWF)-cleaving protease. Low ADAMTS13 plasma levels are associated with an increased risk… read more here.
Keywords: adamts13; expression; endothelial cells; von willebrand ... See more keywords
First use of the anti‐VWF nanobody caplacizumab to treat iTTP in pregnancy
Sign Up to like & getrecommendations! Published in 2021 at "British Journal of Haematology"
DOI: 10.1111/bjh.17833
Abstract: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare thrombotic microangiopathy (TMA), caused by autoantibodies against the metalloproteinase ADAMTS13. Subsequent accumulation of ultra-large von Willebrand factor (VWF) multimers induces platelet agglutination and microvascular thrombosis. Pregnancy is… read more here.
Keywords: adamts13; patient; pregnancy; first use ... See more keywords
Ratio of von Willebrand factor antigen to ADAMTS13 activity is a useful biomarker for acute‐on‐chronic liver failure development and prognosis in patients with liver cirrhosis
Sign Up to like & getrecommendations! Published in 2021 at "Hepatology Research"
DOI: 10.1111/hepr.13743
Abstract: Acute‐on‐chronic liver failure (ACLF) is associated with a high risk of short‐term mortality after progression to multiple organ failure. A disintegrin‐like and metalloproteinase with thrombospondin type‐1 motifs 13 (ADAMTS13) is a metalloproteinase that specifically cleaves… read more here.
Keywords: development; adamts13; acute chronic; failure ... See more keywords
Involvement of ADAMTS13 and von Willebrand factor in thromboembolic events in patients infected with SARS‐CoV‐2
Sign Up to like & getrecommendations! Published in 2020 at "International Journal of Laboratory Hematology"
DOI: 10.1111/ijlh.13244
Abstract: Dear Editors, Since early in 2020, the first people in Europe were infected with the SARS-CoV-2 virus leading to the COVID-2019 pandemic. In March 2020, we admitted the first patients with a SARS-CoV-2 infection in… read more here.
Keywords: hematology; adamts13; activity; sars cov ... See more keywords
A multicenter evaluation of the Technoscreen ADAMTS13 activity semi-quantitative screening test for thrombotic thrombocytopenic purpura diagnosis and exclusion.
Sign Up to like & getrecommendations! Published in 2023 at "International journal of laboratory hematology"
DOI: 10.1111/ijlh.14077
Abstract: INTRODUCTION Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal microangiopathy, with an untreated mortality rate of around 90%. TTP is caused by severe deficiency in ADAMTS13, which results in accumulation of ultra large… read more here.
Keywords: adamts13; test; activity; thrombotic thrombocytopenic ... See more keywords
Improvement of recombinant ADAMTS13 production through a more optimal signal peptide or an N‐terminal fusion protein
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Thrombosis and Haemostasis"
DOI: 10.1111/jth.15819
Abstract: Recombinant human ADAMTS13 (rADAMTS13) is a key protein in fundamental research for investigating its mode of action and the pathophysiology of thrombotic thrombocytopenic purpura (TTP). However, the expression of rADAMTS13 is quite low in mammalian… read more here.
Keywords: recombinant adamts13; adamts13 production; production optimal; production ... See more keywords
Mechanisms of ADAMTS13 regulation
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DOI: 10.1111/jth.15873
Abstract: Recombinant ADAMTS13 is currently undergoing clinical trials as a treatment for hereditary thrombotic thrombocytopenic purpura, a lethal microvascular condition resulting from ADAMTS13 deficiency. Preclinical studies have also demonstrated its efficacy in treating arterial thrombosis and… read more here.
Keywords: mechanisms adamts13; regulation; adamts13 regulation; adamts13 ... See more keywords
Circulating ADAMTS13 Levels Are Associated with an Increased Occurrence of Obstructive Sleep Apnea
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DOI: 10.1155/2022/1504137
Abstract: Background and Aims Obstructive sleep apnea (OSA) is strongly associated with obesity, metabolic diseases, coronary artery disease (CAD), stroke, hypertension, and other disorders. This study assessed the relationship between circulating a disintegrin and metalloprotease with… read more here.
Keywords: sleep apnea; adamts13; presence osa; circulating adamts13 ... See more keywords
Open ADAMTS13 Conformation in Immune-Mediated Thrombotic Thrombocytopenic Purpura Is Induced By Anti-ADAMTS13 Autoantibodies and Corresponds with an Ongoing ADAMTS13 Pathology
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DOI: 10.1182/blood-2018-99-113762
Abstract: Background. Deficient ADAMTS13 activity (TS13:act 50%. However, also iTTP patients in remission with a persistent (50%, 10-50%,50% in 41, 10-50% in 14 and 50%, confirming our previous results. Interestingly, ADAMTS13 was open in 93% and… read more here.
Keywords: adamts13; conformation; speakers bureau; anti adamts13 ... See more keywords
Reduced Cleavage of Von Willebrand Factor By ADAMTS13 Exacerbates Acute Kidney Injury Secondary to Traumatic Injury
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DOI: 10.1182/blood-2020-138508
Abstract: Introduction: Increases in plasma von Willebrand Factor (vWF) antigen levels, accompanied by decreases in its respective metalloprotease, ADAMTS13, have been demonstrated following severe injury. We hypothesized that following traumatic injury, endothelial damage and activation would… read more here.
Keywords: adamts13; traumatic injury; model; injury ... See more keywords