Results 21 to 30 of about 15,834 (256)

Role of ADAMTS13, VWF and F8 genes in deep vein thrombosis.

PLoS ONE, 2021
BackgroundWe previously described the association between rare ADAMTS13 single nucleotide variants (SNVs) and deep vein thrombosis (DVT). Moreover, DVT patients with at least one rare ADAMTS13 SNV had a lower ADAMTS13 activity than non-carriers.AimsTo ...
Maria Teresa Pagliari, Andrea Cairo, Marco Boscarino, Ilaria Mancini, Emanuela Pappalardo, Paolo Bucciarelli, Ida Martinelli, Frits R Rosendaal, Flora Peyvandi   +8 more
doaj   +2 more sources

Inherited ADAMTS13 mutations associated with Thrombotic Thrombocytopenic Purpura: a short review and update

Platelets, 2023
ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein.
Zoe Markham-Lee, Neil V. Morgan, Jonas Emsley   +2 more
doaj   +1 more source

ADAMTS13 activity and inhibitor [PDF]

American Journal of Hematology, 2008
AbstractThrombotic thrombocytopenic purpura (TTP) is often associated with acquired or congenital deficiency of the von Willebrand factor‐cleaving metalloprotease, ADMATS13 (Lammle B et al., J Thromb Haemost 2005;3:1663‐1675; Schneppenheim et al., Blood 2003;101:1845‐1850).
Adriana, Doldan-Silvero, Carlos, Acevedo-Gadea, Clandine, Habib, Jonathan, Freeman, Vandita, Johari   +4 more
openaire   +2 more sources

Phenotypic expression of ADAMTS13 in glomerular endothelial cells.

PLoS ONE, 2011
BackgroundADAMTS13 is the physiological von Willebrand factor (VWF)-cleaving protease. The aim of this study was to examine ADAMTS13 expression in kidneys from ADAMTS13 wild-type (Adamts13⁺/⁺) and deficient (Adamts13⁻/⁻) mice and to investigate the ...
Ramesh Tati, Ann-Charlotte Kristoffersson, Anne-Lie Ståhl, Matthias Mörgelin, David Motto, Simon Satchell, Peter Mathieson, Minola Manea-Hedström, Diana Karpman   +8 more
doaj   +1 more source

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 ...
Veronica DeYoung, Kanwal Singh, Colin A. Kretz   +2 more
openaire   +2 more sources

AFM Imaging Reveals Multiple Conformational States of ADAMTS13

Journal of Biological Engineering, 2019
Background ADAMTS13 (A disintegrin and metalloprotease with a thrombospondin type 1 motif 13) cleaves Von Willebrand factor (VWF) to regulate its size, thereby preventing aberrant platelet aggregation and thrombus. Deficiency of ADAMTS13 caused by either
Shanshan Yu, Wang Liu, Jinhua Fang, Xiaozhong Shi, Jianhua Wu, Ying Fang, Jiangguo Lin   +6 more
doaj   +1 more source

Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure. [PDF]

, 2009
Decreased activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease, was recently reported in cardiovascular diseases and in hepatic failure.
Borgulya, Gábor, Cervenak, László, Förhécz, Zsolt, Gombos, Tímea, Hársfalvi, Jolán, Jánoskuti, Lívia, Kunde, Jan, Makó, Veronika, Papassotiriou, Jana, Pozsonyi, Zoltán, Prohászka, Zoltán   +10 more
core   +1 more source

Bethesda Assay for Detecting Inhibitory Anti-ADAMTS13 Antibodies in Immune-Mediated Thrombotic Thrombocytopenic Purpura

TH Open, 2018
A diagnosis of thrombotic thrombocytopenic purpura (TTP) is confirmed by a severe deficiency (
Chiara Vendramin, Mari Thomas, John-Paul Westwood, Marie Scully   +3 more
doaj   +1 more source

Diagnosis of thrombotic thrombocytopenic purpura

Терапевтический архив, 2020
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction.
G. M. Galstyan, E. E. Klebanova
doaj   +1 more source

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome [PDF]

, 2014
Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment.
Goodship, T, Scully, M
core   +1 more source