An optimized fluorogenic ADAMTS13 assay with increased sensitivity for the investigation of patients with thrombotic thrombocytopenic purpura

• Published in: Journal of thrombosis and haemostasis Vol. 11; no. 8; pp. 1511 - 1518
• Main Authors: Muia, J., Gao, W., Haberichter, S. L., Dolatshahi, L., Zhu, J., Westfield, L. A., Covill, S. C., Friedman, K. D., Sadler, J. E.
• Format: Journal Article
• Language: English
• Published: England Elsevier Limited 01.08.2013
• Subjects: ADAM Proteins - analysis; ADAMTS13 Protein; Anticoagulants - chemistry; Case-Control Studies; Female; Fluorescent Dyes - chemistry; human; Humans; kinetics; Male; Mutation; Peptides - chemistry; Plasma; Plasmids - metabolism; Purpura, Thrombotic Thrombocytopenic - blood; Purpura, Thrombotic Thrombocytopenic - diagnosis; recombinant fusion proteins; Recombinant Fusion Proteins - metabolism; Reproducibility of Results; Sensitivity and Specificity; Substrate Specificity; Thrombocytopenia - blood; Thrombocytopenia - diagnosis; Thrombotic Microangiopathies - blood; Thrombotic Microangiopathies - diagnosis; thrombotic thrombocytopenic purpura; von Willebrand factor; von Willebrand Factor - chemistry; recombinant fusion proteins; ADAMTS13 protein; von Willebrand factor; substrate specificity; thrombotic thrombocytopenic purpura; kinetics; human

Summary Background Most ADAMTS13 assays use non‐physiological conditions (low ionic strength, low pH, barium chloride), are subject to interference from plasma proteins, hemoglobin and bilirubin, and have limited sensitivity, especially for inhibitors. Objectives We addressed these constraints by de...