Results 171 to 180 of about 9,301 (216)

A red blood cell-based antigen delivery system to facilitate T cell epitope presentation to promote peripheral tolerance to ADAMTS13 in immune-mediated thrombotic thrombocytopenic purpura. [PDF]

Res Pract Thromb Haemost
Dierickx M, Groten S, Miranda M, Langerhorst P, van der Zwaan C, Mul E, Hoogendijk AJ, Voorberg J.   +7 more
europepmc   +1 more source

Ethnicity affects relapse-free survival in immunemediated thrombotic thrombocytopenic purpura. [PDF]

Haematologica
Weisinger J, Blanchard F, Suzon B, Deligny C, Fadlallah J, Provôt F, Poullin P, Marie M, Ribes D, Choukroun G, Delmas Y, Azoulay E, Benhamou Y, Grall M, Halimi JM, Le Quintrec M, Servais A, Papo T, Lepart C, Cartery C, Chatelet V, Augusto JF, Ville S, Perez P, Lièvre L, Legendre M, Rumpler A, Hertig A, Rieu V, Jaccard A, Zunic P, Gilardin L, Martis N, Puig SR, Gupte R, Garcia MDMT, Dierickx M, Greco D, Albrecht T, Icleanu AA, Bouzid R, Joly B, Veyradier A, Picod A, Coppo P.   +44 more
europepmc   +1 more source

[Caplacizumab combined with therapeutic plasma exchange, glucocorticoids, and rituximab for refractory thrombotic thrombocytopenic purpura: a case report and literature review]. [PDF]

Zhonghua Xue Ye Xue Za Zhi
Wei QZ, Lu C, Jiang HW, Hu JB, Wu YH, Hu Y.   +5 more
europepmc   +1 more source

Conformational activation of ADAMTS13 [PDF]

Proceedings of the National Academy of Sciences of the United States of America, 2014
Significance We show that a CUB–spacer domain interaction impedes exposure of the ADAMTS13 spacer functional exosite, preventing ADAMTS13 from interacting effectively with its complementary binding site in the VWF A2 domain. This CUB–spacer interaction is disrupted by interaction with the C-terminal domains of VWF, leading to conformational ...
Kieron South, Brenda M Luken, James T B Crawley   +2 more
exaly   +8 more sources

ADAMTS13 and Non-ADAMTS13 Biomarkers in Immune-Mediated Thrombotic Thrombocytopenic Purpura [PDF]

Journal of Clinical Medicine, 2023
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare medical emergency for which a correct and early diagnosis is essential. As a severe deficiency in A Disintegrin And Metalloproteinase with ThromboSpondin type 1 repeats, member 13 (ADAMTS13) is the underlying pathophysiology, diagnostic strategies require timely monitoring of ADAMTS13
Quintijn Bonnez, Kazuya Sakai, Karen Vanhoorelbeke   +2 more
exaly   +5 more sources

Mechanisms of ADAMTS13 regulation

Journal of Thrombosis and Haemostasis, 2022
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 inflammation without causing bleeding, suggesting that ...
Kanwal Singh, Colin A Kretz, Veronica Deyoung   +2 more
exaly   +3 more sources

The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction [PDF]

British Journal of Haematology, 2008
SummaryAutoantibodies to ADAMTS13 (a disintegrin‐like and metalloprotease with thrombospondin type I motif, member 13) play an important role in the development of microthrombosis in thrombotic thrombocytopenic purpura (TTP). In severe cases of antiphospholipid syndrome (APS), microthrombosis can occur similar to that seen in TTP, suggesting possible ...
A S Lawrie, I J Mackie
exaly   +3 more sources