Abstract:
:It has now been 3 years since the von Willebrand factor (VWF)-cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 ADAMTS13 mutations resulting in familial TTP have been reported. Considerable progress has also been realized toward understanding the role of ADAMTS13 in normal hemostasis, as well as the mechanisms by which ADAMTS13 deficiency contributes to TTP pathogenesis. Measurement of ADAMTS13 activity in TTP and other pathologic conditions also remains a focus of a substantial clinical research effort. Building on these studies, continued investigation of ADAMTS13 and VWF holds considerable promise for advancing the understanding of TTP pathogenesis and should lead to improved diagnosis and treatment for this important hematologic disease.
journal_name
Bloodjournal_title
Bloodauthors
Levy GG,Motto DG,Ginsburg Ddoi
10.1182/blood-2004-10-4097subject
Has Abstractpub_date
2005-07-01 00:00:00pages
11-7issue
1eissn
0006-4971issn
1528-0020pii
2004-10-4097journal_volume
106pub_type
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