ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis
ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis
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DOI：
10.1111/j.1365-2141.2007.06688.x
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发表时间：
2007-08-01
影响因子：
6.5
通讯作者：
Mannucci, Pier M.
中科院分区：
文献类型：
--
作者：
Feys, Hendrik B.;Canciani, Maria T.;Mannucci, Pier M.
血浆金属蛋白酶ADAMTS13(一种带有血栓反应蛋白1型基序的去整合素和金属蛋白酶)可将血栓形成前的超长血小板黏附蛋白von Willebrand因子(ULVWF)多聚体切割成活性较低的多聚体，从而促进受损血管的止血。当该酶功能失调或检测不到时，循环中的ULVWF可能会导致大量的血小板血管内聚集和血栓性血小板减少性紫癜。这项研究比较了从具有不同健康和疾病状况的受试者收集的大量血浆中的ADAMTS13抗原和活性，其中大多数与血栓形成倾向增加有关。病理情况：肝硬变90例，炎症性肠病44例，心脏手术30例。健康状况包括妊娠(n=42)、口服避孕药(n=33)和新生儿状态(n=41)。以不同年龄的正常人为对照(n=132)。抗原测定法比基于胶原结合的活性测定法的变异性更小。在正常人中，抗原值与活动度有很好的相关性，但在新生儿、孕妇年龄较晚的妊娠和心脏手术中，抗原值有不同程度的差异。在肝硬变和炎症性肠病中没有发现差异，这两者都与低血浆ADAMTS13水平有关。ADAMTS13活性和抗原的平行测量为理解VWF裂解酶在健康和疾病中的行为提供了新的工具。
The plasma metalloprotease ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin type 1 motif 13) cleaves prothrombotic ultralarge multimers of the platelet-adhesive protein von Willebrand factor (ULVWF) into less active multimers that promote haemostasis in injured blood vessels. When the enzyme is dysfunctional or undetectable, circulating ULVWF may cause massive intravascular aggregation of platelets and thrombotic thrombocytopenic purpura. This study compared ADAMTS13 antigen and activity in a large set of plasmas collected from subjects with various conditions of health and disease, most of which were associated with an increased thrombotic tendency. Pathological conditions were liver cirrhosis (n = 90), inflammatory bowel disease (n = 44) and cardiac surgery (n = 30). Healthy conditions were pregnancy (n = 42), oral contraceptive intake (n = 33) and the neonatal state (n = 41). Normal individuals of different ages were taken as controls (n = 132). The antigen assay showed less variability than the collagen binding-based activity assay. Antigen values correlated well with activity in normal individuals, but were discrepant to various degrees in neonates, pregnancies of later maternal age and cardiac surgery. No discrepancies were noted in liver cirrhosis and inflammatory bowel disease, which were both associated with low-plasma levels of ADAMTS13. The parallel measurement of ADAMTS13 activity and antigen provides a new tool for understanding the behaviour of the VWF cleaving protease in health and disease.