Results 51 to 60 of about 3,354,179 (197)

Deficiency of complement factor H-related proteins and autoantibody-positive hemolytic uremic syndrome in an infant with combined partial deficiencies and autoantibodies to complement factor H and ADAMTS13 [PDF]

Clinical Kidney Journal, 2018
A 3-month-old male infant developed an extremely severe episode of atypical hemolytic uremic syndrome (aHUS) associated with partial deficiencies of full-length complement factor H (FH; ∼15% of infant normal) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) (39% of normal) and autoantibodies reactive with
Mini Michael, Nancy Turner, Ewa Elenberg, Linda G Shaffer, Jun Teruya, Mazen Arar, Shiu-Ki Hui, Richard J Smith, Joel Moake   +8 more
openaire   +3 more sources

The Intriguing Relationships of von Willebrand Factor, ADAMTS13 and Cardiac Disease

Journal of Cardiovascular Development and Disease, 2021
von Willebrand factor (VWF) is an adhesive protein involved in primary hemostasis and facilitates platelet adhesion to sites of vascular injury, thereby promoting thrombus formation. VWF exists in plasma as multimers of increasing size, with the largest (
Benjamin Reardon, Leonardo Pasalic, Emmanuel J. Favaloro   +2 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

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  

Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function [PDF]

, 2020
Plasma ADAMTS13 circulates in a folded conformation that is stabilized by an interaction between the central Spacer domain and the C-terminal CUB (complement components C1r and C1s, sea urchin protein Uegf, and bone morphogenetic protein-1) domains ...
Schelpe, An-Sofie, De Meyer, Simon F., Petri, Anastasis, Pareyn, Inge, Crawley, James TB, Roose, Elien, Vanhoorelbeke, Karen, Deckmyn, Hans, Crawley, James T.B., De Meyer, Simon F   +9 more
core   +1 more source

Thirty five novel nsSNPs may effect on ADAMTS13 protein leading to Thrombotic thrombocytopenic purpura (TTP) using bioinformatics approach [PDF]

, 2019
ABSTRACT Background Genetic polymorphisms in the ADAMTS13 gene are associated with thrombotic thrombocytopenic purpura or TTP, a life-threatening microangiopathic disorder.
Abdelhameed, Tebyan A., Ahmed, Arwa I., Mustafa, Mujahed I., Eltayeb, Amel N., Abdelrhman, Fatima A., Ahmed, Amal B., Ahmed, Najla B., Khadir, Hiba Y., Hassan, Adla M., Mohamed, Huda K., Osman, Soada A., Elhag, Mustafa, Hassan, Mohamed A.   +12 more
openaire   +1 more source

Deficiency of high‐molecular‐weight von Willebrand factor mitigates thrombo‐complement injury in an LPS‐induced TMA‐like mouse model

VIEW, EarlyView.
Reduction of high‐molecular‐weight von Willebrand factor disrupts the platelet–complement amplification loop, attenuating microvascular thrombosis, complement deposition, endothelial injury, and organ damage in complement‐mediated thrombotic microangiopathy.
Yang Li, Chihao Zhang, Xiaoqian Xu, Sicong Wang, Xiaofan Hu, Junjie Yao, Xi Wu, Difeng Zhu, Qian Liang, Yeling Lu, Yu Liu, Guanqun Xu, Jiakuan Zhou, Qiulan Ding, Wenman Wu, Ya Li, Jing Dai, Xuefeng Wang   +17 more
wiley   +1 more source

Von Willebrand Factor and ADAMTS13

, 2011
Objective— The goal of this study was to search for an association between a desintegrin-like and metalloprotease thrombospondin type 1 motif, member 13 (ADAMTS13) levels and the occurrence of preeclampsia, its characteristics ...
Laurent Mandelbrot, Marie Cohen-Moatti, Nejma Ameziane, Thibaut Sanglier, Dominique de Prost, Agnès Veyradier, Paulette Legendre, Alain Stepanian, Vassilis Tsatsaris   +8 more
core   +1 more source

Thrombotic Microangiopathy‐Like Phenotype in Patients With Infection‐Associated Disseminated Intravascular Coagulation Treated With Thrombomodulin Alfa

European Journal of Haematology, EarlyView.
ABSTRACT Despite disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) sharing features of thrombocytopenia, organ dysfunction, and bleeding, the relationship between these two conditions remains unclear. We therefore conducted a post hoc analysis of post‐marketing surveillance data from Japan to evaluate the clinical ...
Naoki Takezako, Fumiyo Komatsu, Goichi Honda, Noriaki Kawano, Toshimasa Uchiyama, Kazuo Kawasugi, Seiji Madoiwa, Kei Suzuki, Yoshinobu Seki, Takayuki Ikezoe, Kohji Okamoto, Hideo Wada   +11 more
wiley   +1 more source

A phenotype–genotype correlation of ADAMTS13 mutations in congenital thrombotic thrombocytopenic purpura patients treated in the United Kingdom

, 2012
Background:  ADAMTS13 mutations play a role in thrombotic thrombocytopenic purpura (TTP) pathogenesis. Objectives:  To establish a phenotype–genotype correlation in a cohort of congenital TTP patients.
Machin, S.J., Camilleri, R., Scully, M., Manns, K., Thomas, M., Mackie, I.J., Chen, W., Liesner, R.   +7 more
core   +1 more source