期刊
PLATELETS
卷 34, 期 1, 页码 -出版社
TAYLOR & FRANCIS INC
DOI: 10.1080/09537104.2022.2138306
关键词
ADAMTS13; Thrombotic Thrombocytopenic Purpura; VWF
ADAMTS13 is a plasma metalloprotease that cleaves VWF to maintain hemostasis, and its mutations are associated with Thrombotic Thrombocytopenic Purpura. Molecular characterization of ADAMTS13 is important for understanding the mechanisms of TTP.
ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein. ADAMTS13 is a multi-domain protein with the domains broadly functioning to interact and cleave VWF or maintain global latency of ADAMTS13. Thrombotic Thrombocytopenic Purpura is a disease characterized by excessive thrombi formation in the microvasculature, diagnosis is made when ADAMTS13 activity is <10%. In the hereditary form, a variety of mutations are found throughout all domains of ADAMTS13, examples are given alongside details of each domain in this article. ADAMTS13 mutations can inhibit the binding and cleavage of VWF directly or indirectly through reduced secretion, leading to increased size of VWF multimers and platelet recruitment. Molecular characterization of ADAMTS13 may provide insight into the mechanisms of TTP to aid in both scientific and clinical research.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据