Relation between ADAMTS13 activity and ADAMTS13 antigen levels in healthy donors and patients with thrombotic microangiopathies (TMA)

Manfred Rieger, Silvia Ferrari, Johanna A. Kremer Hovinga, Christian Konetschny, Andrea Herzog, Letizia Koller, Alfred Weber, Guiseppe Remuzzi, Michael Dockal, Barbara Plaimauer, Friedrich Scheiflinger

Research output: Contribution to journalArticlepeer-review

Abstract

We have established a new, enzyme-linked immunosorbent assay (ELISA) for the detection of ADAMTS 13 antigen using purified polyclonal rabbit anti-human ADAMTS13 IgG. Normal plasma ADAMTS13 antigen levels span a concentration of 740-1420ng/ml (median 1080ng/ml) resulting in an ADAMTS13 activity to antigen ratio of 0.48 to 1.68 U/μg. In a cohort of HUS patients, ADAMTS13 antigen was in the normal range, whereas in hereditary TTP patients antigen levels were low to undetectable, in concordance with severe deficient ADAMTS13 activity. Plasma of acquired TTP patients was found to contain free as well as autoantibody-bound ADAMTS13. We also prevent evidence for circulating anti-ADAMTS13 antibody/ADAMTS13 antigen immune complexes not only in acutely ill or actively treated patients but also in patients who have already achieved clinical remission. This new developed ADAMTS13 antigen ELISA assay allows rapid determination of ADAMTS13 antigen levels in human plasma but is of limited predictive value for the diagnosis or treatment of acquired TTP due to the detection of ADAMTS13 in antibody complexes.

Original languageEnglish
Pages (from-to)212-220
Number of pages9
JournalThrombosis and Haemostasis
Volume95
Issue number2
DOIs
Publication statusPublished - Feb 2006

Keywords

  • ADAMTS13
  • ADAMTS13-antigen
  • ELISA
  • HUS
  • TTP

ASJC Scopus subject areas

  • Hematology

Fingerprint

Dive into the research topics of 'Relation between ADAMTS13 activity and ADAMTS13 antigen levels in healthy donors and patients with thrombotic microangiopathies (TMA)'. Together they form a unique fingerprint.

Cite this