Characterization of coding synonymous and non-synonymous variants in ADAMTS13 using ex vivo and in silico approaches. [PDF]
Synonymous variations, which are defined as codon substitutions that do not change the encoded amino acid, were previously thought to have no effect on the properties of the synthesized protein(s).
Nathan C Edwards+20 more
doaj +1 more source
Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease. [PDF]
BACKGROUND:The zinc metalloprotease ADAMTS13 is a multidomain protein that cleaves von Willebrand Factor (VWF) and is implicated in Thrombotic Thrombocytopenic Purpura (TTP) pathogenesis.
Zuben E Sauna+11 more
doaj +1 more source
The Intriguing Connections between von Willebrand Factor, ADAMTS13 and Cancer
von Willebrand factor (VWF) is a complex and large protein that is cleaved by ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13), and together they serve important roles in normal hemostasis.
Chanukya K Colonne+2 more
semanticscholar +1 more source
Probing ADAMTS13 substrate specificity using phage display. [PDF]
Von Willebrand factor (VWF) is a large, multimeric protein that regulates hemostasis by tethering platelets to the subendothelial matrix at sites of vascular damage.
Karl C Desch+7 more
doaj +1 more source
Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure. [PDF]
Decreased activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease, was recently reported in cardiovascular diseases and in hepatic failure.
Borgulya, Gábor+10 more
core +1 more source
Phenotypic expression of ADAMTS13 in glomerular endothelial cells.
BackgroundADAMTS13 is the physiological von Willebrand factor (VWF)-cleaving protease. The aim of this study was to examine ADAMTS13 expression in kidneys from ADAMTS13 wild-type (Adamts13⁺/⁺) and deficient (Adamts13⁻/⁻) mice and to investigate the ...
Ramesh Tati+8 more
doaj +1 more source
The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin motifs) family [PDF]
The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin motifs) enzymes are secreted, multi-domain matrix-associated zinc metalloendopeptidases that have diverse roles in tissue morphogenesis and patho-physiological remodeling, in ...
A Colige+145 more
core +1 more source
How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome [PDF]
Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment.
Goodship, T, Scully, M
core +1 more source
Ubiquitous microthromboses in the pulmonary vasculature play a crucial role in the pathogenesis of COVID-19 associated acute respiratory distress syndrome (ARDS). Excess of Willebrand factor (vWf) with intravascular multimer formation was identified as a
Felix S. Seibert+13 more
doaj +1 more source
Pathogenicity of anti-ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura. [PDF]
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease in which anti-ADAMTS13 autoantibodies cause severe enzyme deficiency.
Crawley, JT+3 more
core +2 more sources