Reinventing the wheel in ischemic stroke: Novel aspects in ADAMTS13 and von Willebrand axis

A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), is an enzyme that cleaves ultra-large von Willebrand factor (VWF) multimers. ADAMTS13 deficiency leads to thrombotic thrombocytopenic purpura (TTP), a thrombotic microangiopathy characterized by micro-thrombosis in small vessels and target-organ damage. Given that in long-term TTP survivors, reduced ADAMTS13 activity has been associated with the development of major cardiovascular events, the emerging evidence regarding the dysregulation of the ADAMTS13/ VWF axis in the pathogenesis of ischemic stroke (IS), and the unmet need for novel predictive models for IS outcomes and novel targeted therapeutics, we aim to review the current literature to provide an overview of the ongoing research on this field. Our objective is to summarize the evidence from experimental and clinical studies to underline the pathogenetic and therapeutic potential of VWF-ADAMTS13 in IS, while also providing perspectives for future research in this domain.