BACKGROUND AND OBJECTIVES Methylene blue photo-inactivated plasma (MBPIP) has been reported to be less effective than fresh-frozen plasma (FFP) in the treatment of thrombotic thrombocytopenic purpura, which suggests a reduced content of the von Willebrand factor metalloprotease ADAMTS-13 in MBPIP. MATERIALS AND METHODS ADAMTS-13 activity and von Willebrand factor antigen (vWF:Ag) levels were ...

ADAMTS-13 was recently identified as a new hemostatic factor, von Willebrand factor (VWF)–cleaving protease. Either congenital or acquired defects of the enzymatic activity lead to thrombotic thrombocytopenic purpura (TTP). ADAMTS-13 specifically cleaves a peptidyl bond between Y1605 and M1606 in the A2 domain of VWF. Here, we determined the minimal region recognized as a specific substrate by ...

Thrombotic thrombocytopenia purpura (TTP) caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA). Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE). In thi...

Balance between pro-tumor and anti-tumor effects may be affected by molecular interactions within tumor microenvironment. On this basis we searched for molecular partners of ADAMTS-12, a secreted metalloprotease that shows both oncogenic and tumor-suppressive effects. Using its spacer region as a bait in a yeast two-hybrid screen, we identified fibulin-2 as a potential ADAMTS-12-interacting pro...

Members of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) family are known to influence development, angiogenesis, coagulation and progression of arthritis. As proteinases their substrates include the von Willebrand factor precursor and extracellular matrix components such as procollagen, hyalectans (hyaluronan-binding proteoglycans including aggrecan), decorin, fib...

We report a case of thrombotic thrombocytopenic purpura (TTP) in a 60 years-old woman with Sjogren's syndrome. Symptomatology on admission leads to evoke the diagnosis of TTP. Biological results allow to set the diagnosis. Actually, association of haemolytic anaemia, schizocytes and thrombocytopenia are in favour of TTP. Undetectable ADAMTS 13 activity (below 5%) confirms the diagnosis. In cong...

Atherosclerosis is an inflammatory disorder of the vasculature regulated by cytokines. Amongst the cytokines, IL-33 attenuates the development of atherosclerosis in mouse model systems via several mechanisms, including inhibition of macrophage foam cell formation and promotion of a Th1 to Th2 shift. Proteases produced by macrophages, such as matrix metalloproteinases and members of ADAMTS (a di...

Baicalein is a flavone compound isolated from Scutellaria baicalensis Georgi (Huang Qin) which exerts multiple biological effects including anti-inflammatory, anti-apoptotic and anti-tumor. However, there is little known about the effects of baicalein on cathepsins and a disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS) in osteoarthritis (OA). In the present study, we invest...

BACKGROUND Ovarian carcinomas, usually associated with sex hormones dysregulation, are the leading cause of gynecological neoplastic death. In normal ovaries, hormones play a central role in regulating cell proliferation, differentiation, and apoptosis. On the other hand, hormonal alterations also play a variety of roles in cancer. Stimulation by sex hormones potentially affects gene expression...