Results 101 to 110 of about 9,301 (216)

The allosteric activation of ADAMTS13 [PDF]

, 2019
ADAMTS13 regulates the multimeric size of Von Willebrand Factor (VWF) in plasma by specific, shear-dependent proteolysis. Despite circulating in a seemingly proteolytically competent form, and with a very long active plasma half-life, ADAMTS13 is ...
Petri, Anastasis
core   +1 more source

Open ADAMTS13 conformation index predicts earlier relapse in immune-mediated thrombotic thrombocytopenic purpura. [PDF]

BACKGROUND ADAMTS13 adopts an open conformation in immune-mediated thrombotic thrombocytopenic purpura (iTTP) patients in acute phase while being closed in healthy donors.
De Waele, Laure, Joly, Bérangère S, Sinkovits, György, Rossmann, Heidi, Coppo, Paul, Peyvandi, Flora, De Meyer, Simon F, Fijnheer, Rob, Veyradier, Agnès, Agosti, Pasquale, Falter, Tanja, De Meyer, Simon F., Joly, Bérangère S., Prohászka, Zoltán, Mancini, Ilaria, Tersteeg, Claudia, Vanhoorelbeke, Karen, Von Auer, Charis, Lämmle, Bernhard, Inoue, Takashi, Sakai, Kazuya   +20 more
core   +1 more source

Presence of anti-ADAMTS13 antibodies in obesity. [PDF]

, 2012
The low-grade chronic inflammation present in obesity has been recognizedas a riskfactor for thrombosis, atherosclerosis and cardiovascular complications.
Dal Prà C, Zanato V, Granzotto M, Fabris R, Sartori MT, PLEBANI, MARIO, Vettore S, FABRIS, FABRIZIO, Scarparo P, Foletto M, Lombardi AM, Scarda A, Serra R, Berti De Marinis G, VETTOR, ROBERTO   +14 more
core   +1 more source

Three disintegrin-like domain mutations of ADAMTS13： functional deficiency and association with thrombosis [PDF]

Zhenduanxue lilun yu shijian
Objective To analyze the genotypic and phenotypic characteristics of four thrombotic patients carrying heterozygous mutations in the disintegrin-like domain of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13), and to ...
LIN Liya, WU Xi, MAO Yinqi, CHEN Guangming, WU Wenman, DAI Jing, WANG Xuefeng, DING Qiulan
doaj   +1 more source

A new name in thrombosis, ADAMTS13 [PDF]

Proceedings of the National Academy of Sciences, 2002
Thrombotic thrombocytopenic purpura (TTP) is a rapidly fatal illness, first described by Moschowitz in 1924 (1), that is characterized by anemia, a low platelet count (thrombocytopenia), and microvascular thrombosis. The anemia is caused by hemolysis and is associated with sharply pointed red cell fragments, termed schistocytes, that look as though ...
openaire   +2 more sources

ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. [PDF]

, 2008
BACKGROUND:From 20 to 50% of patients who survive an acute episode of the acquired form of thrombotic thrombocytopenic purpura relapse but clinical and laboratory markers of recurrence are not well established.DESIGN AND METHODS:In 109 patients enrolled ...
Palla, Roberta, Lavoretano, Silvia, D. Mikovic, R. Palla, Canciani, Maria Teresa, Peyvandi, Flora, Mikovic, Danijela, K. Vanhoorelbeke, FABRIS, FABRIZIO, S. Lavoretano, P.M. Mannucci, PALLA R, Laurenti, Luca, M. Karimi, Karimi, Mehran, LAVORETANO S, Feys, Hendrik, Réti, Marienn, M. Réti, KARIMI M, L. Laurenti, VALSECCHI C, Giuffrida, Gaetano, BATTAGLIOLI T, BATTAGLIOLI, TULLIA CARLA ENRICA, FEYS HB, S. Zver, F. Fabris, Battaglioli, Tullia, H. B. Feys, CANCIANI MT, MANNUCCI PM, M. T. Canciani, ZVER S, C. Valsecchi, PEYVANDI F, Valsecchi, Carla, Zver, Samo, RETI M, G. Giuffrida, VANHOORELBEKE K, Vanhoorelbeke, Karen, Mannucci, Pier Mannuccio, LAURENTI L, GIUFFRIDA G, MIKOVIC D, F. Peyvandi   +46 more
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Degradation of two novel congenital TTP ADAMTS13 mutants by the cell proteasome prevents ADAMTS13 secretion [PDF]

, 2016
INTRODUCTION: Over 150 mutations have been identified in the ADAMTS13 gene in patients with congenital thrombotic thrombocytopenic purpura (TTP). The majority of these (86%), lead to reduced (
Mackie, I, Garagiola, I, Machin, S, Underwood, M, Peyvandi, F   +4 more
core  

Conformational quiescence of ADAMTS13 prevents proteolytic promiscuity [PDF]

, 2016
Background Recent work has revealed that ADAMTS13 circulates in a ‘closed’ conformation, only fully interacting with VWF following a conformational change.
Lane, D A, Freitas, M O, Lane, DA, South, K, Freitas, MO, South, K; id_orcid   +5 more
core   +1 more source

Increased expression of ADAMTS13 mRNA correlates with ischemic cerebrovascular disease in systemic lupus erythematosus patients

SAGE Open Medicine, 2013
Objective: We investigated ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13) messenger RNA levels as a biomarker of disease features in systemic lupus erythematosus.
Consuelo M López De Padilla, Molly S Hein, Cynthia S Crowson, Christopher S Choo, Abigail B Green, Michelle Petri, Hatice Bilgic, Emily C Baechler, Ann M Reed   +8 more
doaj   +1 more source

The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura

Haematologica, 2009
The inherited deficiency of ADAMTS13 is usually associated with severe forms of thrombotic thrombocytopenic purpura. Among the mutations identified in the ADAMTS13 gene, none have been described on the TSP1-6 repeat domain.
Roberta Palla, Silvia Lavoretano, Rossana Lombardi, Isabella Garagiola, Mehran Karimi, Abdolreza Afrasiabi, Mani Ramzi, Raimondo De Cristofaro, Flora Peyvandi   +8 more
doaj   +1 more source