New treatment for TTP?

PubMed, the internet portal of biomedical and life sciences literature, indexed an interesting study about a novel therapeutic strategy for TTP (J Biol Chem. 2005 Oct 3 ). Authors are Zhou W, Dong L, Ginsburg D et al., from the Division of Hematology, Montefiore Medical Center, Bronx, NY, USA. A deficiency of the protease, due to mutations in the ADAMTS13 gene or presence of antibodies that inhibit the activity of the protease, causes thrombotic thrombocytopenic purpura (TTP). Plasma therapy, the conventional therapy for TTP, may cause serious adverse reactions and is ineffective in some patients. In order to develop new strategies for improving the diagnosis and treatment of TTP, were produced a series of truncated ADAMTS13 protein and analyzed their binding with and suppression by the IgG derived from the TTP patients. The results revealed that truncation of the ADAMTS13 protein at its cysteine-rich region eliminated its recognition by the antibodies without abolishing its VWF-cleaving activity. This raises the possibility that resistant ADAMTS13 variants may be exploited to circumvent inhibitory antibodies that cause TTP. To access the full abstract of the text study, click here.