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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. +8 more
openaire +2 more sources
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 +6 more
doaj +1 more source
PROTEOLYTIC PROCESSING OF VON WILLEBRAND FACTOR BY ADAMTS13 AND LEUKOCYTE PROTEASES-rev1-MJHID-2013
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
ADAMTS13 in arterial thrombosis [PDF]
The aim of this thesis was to study the role of ADAMTS13 in arterial thrombosis, a disease that comprises both environmental and genetic factors. We determined ADAMTS13 in ischemic stroke patients and controls.
Bongers, T.N. (Tamara)
core
Specific activity of ADAMTS13 variants and limited proteolysis with trypsin.
A. Specific activity of twelve ADAMTS13 variants vs. WT as determined using FRETS-VWF73 substrate: Samples containing equal amounts of total extracellular protein were incubated with 5 µM of FRETS-VWF73 substrate and fluorescence readings were taken at 5
Anton A. Komar (154543) +20 more
core +1 more source
The multidomain metalloprotease ADAMTS13 limits thrombus formation via the cleavage of large multimeric forms of von Willebrand factor. Deficiency of functional ADAMTS13 is associated with a number of disease pathologies including thrombotic ...
Chava Kimchi-Sarfaty, Sandra Chang Tseng
core +1 more source
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 +4 more
doaj
Recognizing thrombotic thrombocytopenic purpura beyond the pentad. [PDF]
Pupiec OM +3 more
europepmc +1 more source
Endotheliopathy in CAR T-Cell Therapy: Mechanistic Insights into the VWF/ADAMTS13 Axis and the Angiopoietin-Tie2 Pathway. [PDF]
de Bruijn S +3 more
europepmc +1 more source
Acquired immune-mediated thrombotic thrombocytopenic purpura with severe cholestatic liver injury and reversible acute kidney injury after ivonescimab-containing chemoimmunotherapy: a case report and literature review. [PDF]
Chen Q, Song X, Zhang M, Niu H, Guo W.
europepmc +1 more source

