title: ADAMTS13--more than just TMA and TTP.
abstract: ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type-1 motifs 13) has been shown to be of major pathophysiological importance for thrombotic microangiopathy (TMA) in the setting of thrombocytic thrombocytopenic purpura (TTP) when either lacking (inherited TTP) or if antibodies against ADAMTS13 are present (acquired TTP). A potential pathophysiological role of ADAMTS13 has also been postulated in other diseases i.e. myocardial infarction, atrial fibrillation and diabetic angiopathy. Recent data provides evidence, however, for a completely different role of ADAMTS13 in vascular physiology and pathophysiology. In the present issue of Nephrology Dialysis and Transplantation, Bockmeyer et al. documented a physiological expression of ADAMTS13 in arteriolar vascular smooth muscle cells (VSMCs) which is maintained in the early, so-called benign nephrosclerosis, i.e. under conditions of early hypertensive vascular damage, and is completely lost if the damage proceeds to late, fibrotic stages of vascular injury. Thus, the recent report identifies ADAMTS13 as a new marker for the contractile phenotype of VSMC which is maintained in the early, hyalinotic phase of vascular damage, but is lost in later, more fibrotic stages of benign nephrosclerosis. This finding adds a novel and potentially important aspect to the unsolved and underestimated problem of benign or hypertensive nephrosclerosis.
authors.forename: Kerstin
authors.initials: K
authors.lastname: Amann
mesh_terms: D051722:ADAM Proteins / Q000235:genetics / Q000378:metabolism*; D000071120:ADAMTS13 Protein; D015415:Biomarkers / Q000378:metabolism*; D006801:Humans; D009400:Nephrosclerosis / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D011379:Prognosis
chemical_list: D015415:Biomarkers; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human