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1.  ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura 
European Journal of Pediatrics  2006;166(3):249-257.
The activity of ADAMTS13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n = 20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS13 was not detected in the plasma from patients with congenital TTP (n = 5) by either antibody, whereas patients with acquired TTP (n = 2) presented the normal phenotype. Following immunoadsorption of immunoglobulins, the ADAMTS13 band was removed from the plasma of the patients with acquired TTP, but not from that of normal individuals. This indicates that ADAMTS13 is complexed with immunoglobulin in these patients. The lack of ADAMTS13 expression in the plasma from patients with hereditary TTP may indicate defective synthesis, impaired cellular secretion, or enhanced degradation in the circulation. This study differentiated between normal and TTP plasma, as well as between congenital and acquired TTP. This method may, therefore, be used as a complement in the diagnosis of TTP.
PMCID: PMC1820762  PMID: 17187257
ADAMTS13; von Willebrand factor; Thrombotic thrombocytopenic purpura; Immunoblotting; Plasma; von Willebrand factor cleaving protease
2.  Acute Phase Protein Response and Polymorphonuclear Leukocyte Cathepsin G Release After Slow Interleukin-1 Stimulation in the Rat 
Mediators of Inflammation  1994;3(6):425-431.
In this work we have studied the acute phase protein response and degranulation of polymorphonuclear leukocytes in vivo in the rat after a slow interleukin-1β stimulation. A total dose of 1 μg, 2 μg, 4 μg and 0 μg (controls with only vehicle) of interleukin-1β was released from osmotic minipumps over a period of 7 days. The pumps were implanted subcutaneously. A cystic formation was formed around the pumps that contained interleukin-1β whereas no tissue reaction was seen around pumps containing only vehicle. Besides flbroblasts the cyst wall contained numerous polymorphonuclear leukocytes which were positively stained for cathespin G. α2-macroglobulin, α1-inhtbitor-3, α1-proteinase inhibitor, albumin and C3 were measured by electroimmunoassay and all showed plasma concentration patterns that were dose-dependent to the amount of interleuktn-1β released. Fibrinogen in plasma was elevated in the control group but showed decreased plasma values with higher doses of interleukin-1β released. All animals showed increased plasma levels of cathespin G but the lowest levels for cathespin G were seen for the highest interleukin-1β dose released. It was clearly seen that a slow continuous release of interleukin-1β in vivo caused an inflammatory reaction. Plasma levels for the proteins analysed all showed a similar pattern, namely an initial increase or decrease of plasma concentration followed by a tendency to normalization of plasma values. It was concluded that a long-term interleukin-1β release could not sustain the acute phase protein response elicited by the initial interleukin-1β release.
PMCID: PMC2365584  PMID: 18475591

Results 1-2 (2)