Results 71 to 80 of about 5,761 (161)

Unraveling antibody-induced structural dynamics in the ADAMTS13 CUB1-2 domains via HDX-MS

Blood Advances
: Allosteric regulation of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type-1 motif, member 13) activity involves an interaction between its spacer (S) and 2 complement C1r/C1s, Uegf and BMP1 (CUB; CUB1-2) domains to keep the enzyme
Quintijn Bonnez, Emily R. Legan, Inge Pareyn, Febe Boudry, Jennifer Anckaert, Claudia Tersteeg, Simon F. De Meyer, Renhao Li, Karen Vanhoorelbeke   +8 more
doaj   +1 more source

Successful Treatment of Refractory Immune Thrombotic Thrombocytopenic Purpura with Daratumumab

Клиническая онкогематология
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening disease manifested by thrombocytopenia, microangiopathic hemolytic anemia, and impaired functions of organs and systems due to the thrombosis of small arteries.
E. E. Klebanova, Gennadii Martinovich Galstyan, S. Yu. Mamleeva, D. K. Bessmertnyy, Z. T. Fidarova, E. N. Parovichnikova   +5 more
doaj   +1 more source

Moschcowitz Disease in pediatric. [PDF]

Batna Journal of Medical Sciences
Hemichorea Moskowitz disease is a rare hematological disorder known to be an autoimmune disease in most patients. Most cases of thrombotic thrombopenic purpura (TTP) are caused by problems with an enzyme or protein in the blood called ADAMTS13.
Samira AGGOUNE, Mohamed Faouzi SEDDIKI
doaj   +1 more source

Thrombotic thrombocytopenic purpura: Description and analysis of 23 cases treated in Chile between 2017 and 2022

Medwave
Introduction Since the first description of Thrombotic Thrombocytopenic Purpura, caused by a severe deficiency of ADAMTS13, plasma exchange and immunosuppression have become standard treatments, allowing to decrease its high mortality rate.
Daniel Cathalifaud, Jean-Paul Manríquez, Benjamín Rodríguez, Gonzalo Eymin, Benjamín Sanfuentes, Joel Castellano, Andrés Valenzuela   +6 more
doaj   +1 more source

Thrombotic Thrombocytopenic Purpura in Oman: Disease Burden and Outcomes

Oman Medical Journal
Objectives: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening autoimmune disorder; limited information about this disease is available from the Middle East.
Samata Al Dowaiki, Khalid Al Hashmi, Sulayma Al-Lamki, Muhana Al Muselhi, Murtadha Al-Khabori   +4 more
doaj   +1 more source

Can Plasma ADAMTS13 Differentiate Patients With Pulmonary Arterial Hypertension From Other Forms of Pulmonary Hypertension and Dyspnea Control PatientsTake-Home Points

CHEST Pulmonary
Background: Multimarker panels of blood-borne biomarkers could aid in shortening the diagnostic delay of pulmonary arterial hypertension (PAH). Research Question: Can any of the 61 proteins included in the study, related to pathways known to be involved ...
Kriss Kania, MD, Abdulla Ahmed, MD, Salaheldin Ahmed, MD, Adam Engel Sällberg, MD, Karin Tran-Lundmark, MD, PhD, Jørn Carlsen, MD, PhD, Göran Rådegran, MD, DMSc, MSc Eng Phys   +6 more
doaj   +1 more source

[Management of congenital thrombotic thrombocytopenic purpura in the era of recombinant ADAMTS13 protein: Recommendations from the Reference Center for Thrombotic Microangiopathies (CNR-MAT)].

La Revue de medecine interne
Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by a severe deficiency of ADAMTS13, the specific protease that cleaves von Willebrand factor. The congenital form of TTP (cTTP) results from pathogenic variants of the ADAMTS13 gene.
Joly, Bérangère S., Veyradier, Agnès, Coppo, Paul, Joseph, Adrien, Dossier, Claire, Kwon, Theresa, Gouge-Biebuyck, Nathalie, Boyer, Olivia, Tsatsaris, Vassilis   +8 more
openaire   +2 more sources

PROTEOLYTIC PROCESSING OF VON WILLEBRAND FACTOR BY ADAMTS13 AND LEUKOCYTE PROTEASES-rev1-MJHID-2013

Mediterranean Journal of Hematology and Infectious Diseases, 2014
ADAMTS13 is a 190 kDa zinc protease encoded by a gene located on chromosome 9q34.   This protease specifically hydrolyzes von Willebrand factor (VWF) multimers, thus causing VWF size reduction.
Raimondo De Cristofaro
doaj  

Poster Sessions

HemaSphere, Volume 10, Issue S1, June 2026.
wiley   +1 more source

Púrpura trombocitopênica trombótica: o papel do fator von Willebrand e da ADAMTS13 Thrombotic thrombocytopenic purpura: the role of von Willebrand factor and ADAMTS13

Revista Brasileira de Hematologia e Hemoterapia, 2010
A púrpura trombocitopênica trombótica (PTT) instala-se de modo abrupto e é caracterizada pela oclusão difusa de arteríolas e capilares da microcirculação, levando à isquemia de tecidos.
Leandro C. Tonaco, Danyelle R. A. Rios, Lauro M. Vieira, Maria das Graças Carvalho, Luci M. S. Dusse   +4 more
doaj