GC1126A, a novel ADAMTS13 mutein, evades autoantibodies in immune-mediated thrombotic thrombocytopenic purpura [PDF]
Scientific ReportsImmune-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 +13 more
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A Comparative Analysis of the Roles of von Willebrand Factor and ADAMTS13 in Hepatocellular Carcinoma: A Bioinformatics and Microarray-Based Study [PDF]
Current Issues in Molecular BiologyGenetic and epigenetic alterations of various biomolecules at the molecular level can contribute to the pathogenesis of hepatocellular carcinoma (HCC) and negative impact prognosis.
Durmuş Ayan +5 more
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Localization of blood proteins thrombospondin1 and ADAMTS13 to cerebral corpora amylacea [PDF]
Neuropathology, 2009 Corpora amylacea (CA) have long been described in aging brains and in patients with neurodegenerative conditions, but their origins have been debated. It has been proposed that CA represent collections of nervous system breakdown products that accumulate within astrocytic cytoplasm.
Meng, He +3 more
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An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimers [PDF]
Blood, 2007 AbstractSevere deficiency of ADAMTS13, a von Willebrand factor (VWF)–cleaving metalloprotease, causes thrombotic thrombocytopenic purpura. When analyzed with VWF multimers, but not with an abbreviated VWF peptide (VWF73) as the substrate, the plasma ADAMTS13 activity levels of mouse strains segregated into a high and a low group that differed by ...
Han-Mou Tsai +2 more
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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
Shiu Ki Hui +13 more
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Platelets, 2023 ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein.
Zoe Markham-Lee +2 more
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Characterization of ADAMTS13 and von Willebrand factor levels in septic and non-septic ICU patients.
PLoS ONE, 2021 Sepsis is a life-threatening disease characterized by excessive host response to infection that can lead to activation of the coagulation system. Von Willebrand Factor (VWF) and ADAMTS13 are important regulators of hemostasis and their dysregulation ...
Kanwal Singh +7 more
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Escherichia coli-derived von Willebrand factor-A2 domain fluorescence/Förster resonance energy transfer proteins that quantify ADAMTS13 activity [PDF]
Analytical Biochemistry, 2011 The cleavage of the A2 domain of von Willebrand factor (VWF) by the metalloprotease ADAMTS13 regulates VWF size and platelet thrombosis rates. Reduction or inhibition of this enzyme activity leads to thrombotic thrombocytopenic purpura (TTP). We generated a set of novel molecules called VWF-A2 FRET (fluorescence/Förster resonance energy transfer ...
Kannayakanahalli M. Dayananda +2 more
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Chinese Journal of Physiology, 2023 Deep vein thrombosis (DVT) is a common complication in hematologic malignancies and immunologic disorders. Endothelial cell injury and dysfunction comprise the critical contributor for the development of DVT.
Guangfeng Zheng +7 more
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Von Willebrand factor, ADAMTS13 and mortality in dialysis patients
BMC Nephrology, 2021 Background Von Willebrand Factor (VWF) multimers are cleaved into smaller and less coagulant forms by the metalloprotease ADAMTS13. The aim of this study was to investigate the association between VWF and ADAMTS13 and mortality in dialysis patients ...
Gurbey Ocak +8 more
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