ADAMTS13 antibody and inhibitor assays

Book chapter

Moore, G., Vetr, H. and Binder, N. 2023. ADAMTS13 antibody and inhibitor assays. in: Favaloro, E. and Gosselin, R. (ed.) Hemostasis and Thrombosis: Methods and Protocols New York, NY Humana.
Chapter titleADAMTS13 antibody and inhibitor assays
AuthorsMoore, G., Vetr, H. and Binder, N.

A finding of an ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13) activity level of <10% of normal is usually sufficient to distinguish thrombotic thrombocytopenic purpura (TTP) from other thrombotic microangiopathies. TTP can be congenital or acquired, the most common form being acquired immune-mediated TTP caused by autoantibodies than inhibit ADAMTS13 function and/or increase its clearance. Basic 1 + 1 mixing tests can detect the presence of inhibitory antibodies, and quantification can be achieved with Bethesda-type assays that measure loss of function in a series of mixtures of test plasma and normal plasma. Not all patients present with inhibitory antibodies, and here the ADAMTS13 deficiency may be caused by clearing antibodies alone, which are not detectable in functional assays. ELISA assays are commonly used to detect clearing antibodies via capture with recombinant ADAMTS13. Since they also detect inhibitory antibodies, they are the preferred assay, although they cannot distinguish between inhibitory and clearing antibodies. The present chapter describes principles, performance, and practical aspects of a commercial ADAMTS13 antibody ELISA and a generic approach to Bethesda-type assays for detecting inhibitory ADAMTS13 antibodies.

KeywordsAutoantibodies, ADAMTS13 Protein, ADAM Proteins, Plasma, ADAMTS13, Thrombotic microangiopathy, Humans, Rapid assay, Bethesda-type assay, ELISA, Fluorescence resonance energy transfer assay, Thrombotic thrombocytopenic purpura, Inhibitor, Purpura, Thrombotic Thrombocytopenic - diagnosis
Sustainable Development Goals3 Good health and well-being
Middlesex University ThemeHealth & Wellbeing
Book titleHemostasis and Thrombosis: Methods and Protocols
EditorsFavaloro, E. and Gosselin, R.
Place of publicationNew York, NY
SeriesMethods in Molecular Biology (Clifton, N.J.)
Publication dates
Online20 May 2023
Print20 May 2023
Publication process dates
Deposited01 Jun 2023
Output statusPublished
Digital Object Identifier (DOI)
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