Results 91 to 100 of about 10,178 (166)

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

A Pathophysiological Role for ADAMTS13 in Thrombotic Thrombocytopenic Purpura (TTP) without Severe ADAMTS13 Deficiency.

Background: A severe ADAMTS13 deficiency (<5% of the normal) is a specific finding for an acute thrombotic microangiopathy commonly labeled as idiopathic TTP. However, the sensitivity of this finding for the clinical diagnosis of idiopathic TTP is
Lammle, Bernhard, Terrell, Deirdre R., Hovinga, Johanna A. Kremer, Vesely, Sara K., Budde, Ulrich, Sulzer, Irmela, George, James N.   +6 more
core   +1 more source

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  

ADAMTS13 assays in thrombotic microangiopathy

, 2012
Thrombotic microangiopathy is featured by microangiopathic haemolytic anaemia, thrombocytopenia and the presence of peripheral fragmented red cells. Thrombotic thrombocytopenic purpura (TTP) is the major disease entity of concern, which is caused by a ...
Lam, Wang-hoi., 林宏凱.
core   +1 more source

IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura

, 2009
BACKGROUND: ADAMTS13-neutralizing IgG autoantibodies are the major cause of acquired thrombotic thrombocytopenic purpura (TTP). OBJECTIVE: To analyze the IgG subclass distribution of anti-ADAMTS13 antibodies and a potential relationship between subclass ...
Rieger, M, Ferrari, S, Kremer Hovinga, J A, Veyradier, A, Scheiflinger, F, Mudde, G C   +5 more
core   +1 more source

Acquired TTP: ADAMTS13 meets the immune system

, 2014
The majority of the patients affected by acquired thrombotic thrombocytopenic purpura (TTP) develop autoantibodies directed towards ADAMTS13 that interfere with its von Willebrand Factor (VWF) processing activity.
Sorvillo, Nicoletta, Voorberg, Jan, Verbij, Fabian C., Fijnheer, Rob   +3 more
core   +1 more source

Presence of anti-ADAMTS13 antibodies in obesity.

, 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

GC1126A, a novel ADAMTS13 mutein, evades autoantibodies in immune-mediated thrombotic thrombocytopenic purpura

Scientific Reports
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening blood disorder characterized by the formation of blood clots in small blood vessels. It is caused by antibodies targeting the A disintegrin and metalloprotease with
Heechun Kwak, Gahee Choi, Suyong Kim, Ji-Min Park, Youngeun Kwon, Yongmin Lee, Chaemok Lee, Shangbin Yang, Spero Cataland, Sunghyun Kim, Soo-Mee Bang, Jae-Ho Yoon, Wooin Lee, Hyun-Ja Nam   +13 more
doaj   +1 more source

Evaluation of von Willebrand factor in Sickle Cell Disease at Steady State and in Vaso-occlusive Crisis

Medical Journal of Babylon
Background: Sickle cell disease (SCD) is the most common inherited hemoglobinopathy, involved the presence of hemoglobin S in red cells resulting from point mutations, causing replacement of glutamic acid by valine at position 6 of the beta-globin chain.
Huda Abedalameir Hussain, Zainab Wahab Al Maaroof, Abbas Fadhil Hasson   +2 more
doaj   +1 more source

Thrombotic thrombocytopenic purpura associated with pegylated-interferon alpha-2a by an ADAMTS13 inhibitor in a patient with chronic hepatitis C

Haematologica, 2006
A deficiency of ADAMTS13 leads to platelet clumping and/or thrombi formation, finally resulting in thrombotic thrombocytopenic purpura (TTP). In this study, a 62-year-old male with chronic hepatitis C developed TTP a month after long-term pegylated ...
K Kitano, Y Gibo, A Kamijo, K Furuta, S Oguchi, S Joshita, Y Takahashi, F Ishida, M Matsumoto, M Uemura, Y Fujimura   +10 more
doaj