Zebrafish model reveals developmental and hematopoietic functions of ADAMTS13.

ABSTRACT: ADAMTS13 is a metalloprotease that cleaves the von Willebrand factor and prevents pathological thrombosis. Severe genetic deficiency of ADAMTS13 causes congenital thrombotic thrombocytopenic purpura, a life-threatening thrombotic microangiopathy. Increasing evidence suggests that ADAMTS13 contributes to physiological processes beyond hemostasis, including vascular development and tissue homeostasis, but these functions remain poorly understood. To address this gap, we generated a transparent, multitransgenic adamts13i5 zebrafish model and began investigating the developmental and disease-related roles of ADAMTS13 in vivo. The adamts13i5 mutants recapitulated hallmark features of congenital thrombotic thrombocytopenic purpura, including erythrocyte fragmentation and schistocyte formation in

SUBMITTER: Sartori S 

PROVIDER: S-EPMC12584400 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature