Results 51 to 60 of about 9,301 (216)

Special delivery for ADAMTS13 [PDF]

Blood, 2015
In this issue of Blood, using a transgenic mouse model, Pickens et al describe that ectopic expression of ADAMTS13, exclusively in platelets, is able to provide effective protection in murine models of arterial thrombosis and thrombotic thrombocytopenic purpura (TTP ...
openaire   +2 more sources

The relationship between ADAMTS13 genotype and phenotype in congenital thrombotic thrombocytopenic purpura and characterisation of ADAMTS13 mutants [PDF]

, 2015
Congenital thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, usually involving ADAMTS13 gene defects. ADAMTS13 processes the multimeric plasma glycoprotein Von Willebrand factor making it less reactive to platelets.
Underwood, MI
core  

Novel cryptic ADAMTS13 epitopes uncover a distinct open ADAMTS13 conformation in immune-mediated TTP

Haematologica
Open ADAMTS13 conformation is gaining clinical interest as a biomarker for diagnosing immune-mediated thrombotic thrombocytopenic purpura (iTTP) and monitoring remission patients for increased relapse risks.
Quintijn Bonnez, Febe Boudry, Laure De Waele, Lisa Vermeersch, Kadri Kangro, Inge Pareyn, Edwige Tellier, Gilles Kaplanski, Claudia Tersteeg, Bérangère S. Joly, Paul Coppo, Agnès Veyradier, Simon F. De Meyer, Karen Vanhoorelbeke   +13 more
doaj   +1 more source

Recombinant ADAMTS13 in Congenital Thrombotic Thrombocytopenic Purpura. [PDF]

BACKGROUND Congenital thrombotic thrombocytopenic purpura (TTP) results from severe hereditary deficiency of ADAMTS13. The efficacy and safety of recombinant ADAMTS13 and standard therapy (plasma-derived products) administered as routine prophylaxis ...
Xiao, Shan, Cataland, Spero R, Kremer Hovinga, Johanna A, López-Fernández, M Fernanda, Hassenpflug, Wolf-Achim, Zhang, Pinghai, Scully, Marie, Ortel, Thomas L, Coppo, Paul, Dossier, Claire, Bhattacharya, Indranil, Cronin, Michael, Matsumoto, Masanori, Mellgård, Björn, Patwari, Parth, Patel, Munjal, Li, Hong, Biebuyck, Nathalie, Kentouche, Karim, Knöbl, Paul, Antun, Ana, Windyga, Jerzy, Wang, Linda T   +22 more
core   +1 more source

The emerging concept of residual ADAMTS13 activity in ADAMTS13-deficient thrombotic thrombocytopenic purpura [PDF]

, 2013
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread microvascular thrombosis. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is ...
H. M. Wu, L. A. Lotta, K. M. Musallam, F. Peyvandi   +3 more
core   +1 more source

Diagnosis and follow‐up of thrombotic thrombocytopenic purpura with an automated chemiluminescent ADAMTS13 activity immunoassay

Research and Practice in Thrombosis and Haemostasis, 2021
Background Thrombotic thrombocytopenic purpura (TTP) is a life‐threatening thrombotic microangiopathy (TMA) caused by a severe functional deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type I repeats‐13), the specific von ...
Nicolas Beranger, Sandrine Benghezal, Bérangère S. Joly, Sophie Capdenat, Adeline Delton, Alain Stepanian, Paul Coppo, Agnès Veyradier   +7 more
doaj   +1 more source

Perinatal outcomes in normotensive versus hypertensive HELLP syndrome

International Journal of Gynecology &Obstetrics, EarlyView.
Abstract Objective To compare maternal and neonatal outcomes between women with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome with and without associated hypertension. Methods This was a retrospective cohort study at a tertiary university‐affiliated medical center including all singleton HELLP cases >24 weeks gestation (January ...
Matan Anteby, Keren‐Or Wertheimer, Omri Dominsky, Liran Hiersch, Yariv Yogev   +4 more
wiley   +1 more source

Development of a protease-resistant ADAMTS13 to improve stability against proteolytic degradation

Blood Advances
: Recombinant ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was recently approved by the US Food and Drug Administration for the treatment of heritable thrombotic thrombocytopenic purpura, and preclinical ...
Veronica DeYoung, Rex Huang, Hasam Madarati, Rida Malik, Peter Andrisani, Cherie Teney, Colin A. Kretz   +6 more
doaj   +1 more source

Seventh Åland Island Meeting on von Willebrand Disease

Haemophilia, EarlyView.
ABSTRACT Introduction The seventh Åland Island Meeting on von Willebrand Disease (VWD) was held on the Åland archipelago in Finland, from 26 to 28 September 2024. Aim The meeting brought together experts in the field of VWD from around the world to share the latest advances and knowledge in VWD.
Riitta Lassila, Ruben Bierings, Nathan T. Connell, Fernando F. Corrales‐Medina, Augusto B. Federici, Veronica H. Flood, Floor Heubel‐Moenen, Jill M. Johnsen, Geoffrey Kuppens, Davide Matino, Carolyn Millar, Ingmar Schoen, Anish Sharda, Robert F. Sidonio Jr, Alok Srivastava, Timea Szanto, Angela C. Weyand, Erik Berntorp   +17 more
wiley   +1 more source

The role of von Willebrand factor and its cleaving protease, ADAMTS13, in young patients with HIV-related stroke. [PDF]

, 2013
Includes abstract.Includes bibliographical references.The Human Immunodeficiency Virus (HIV) is neuro-invasive and neurological complications of HIV infection occur frequently through a variety of possible mechanisms.
Allie, Sameera
core