Anticoagulant SERPINs: Endogenous Regulators of Hemostasis and Thrombosis.
Anticoagulant SERPINs: Endogenous Regulators of Hemostasis and Thrombosis.
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DOI：
10.3389/fcvm.2022.878199
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发表时间：
2022
影响因子：
3.6
通讯作者：
中科院分区：
文献类型：
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作者：
适当的凝血激活需要血液中促凝和抗凝蛋白之间的平衡。失去这种平衡会导致出血和血栓形成。许多内源性抗凝血蛋白，如抗凝血酶和肝素辅助因子II，都是丝氨酸蛋白酶抑制剂（SERPIN）家族的成员。这些SERPIN抗凝血剂通过与靶凝血蛋白酶形成不可逆的抑制复合物发挥作用。SERPIN家族成员的突变，如抗凝血酶，可引起遗传性血栓症。此外，低血浆SERPINs水平与血栓形成风险增加有关。在此，我们综述了不同抗凝血serpin的生物活性。我们进一步考虑SERPIN缺陷的临床后果和从临床前疾病模型中获得的见解。最后，我们讨论了工程SERPINs作为治疗血栓性病理的新疗法的潜在效用。
Appropriate activation of coagulation requires a balance between procoagulant and anticoagulant proteins in blood. Loss in this balance leads to hemorrhage and thrombosis. A number of endogenous anticoagulant proteins, such as antithrombin and heparin cofactor II, are members of the serine protease inhibitor (SERPIN) family. These SERPIN anticoagulants function by forming irreversible inhibitory complexes with target coagulation proteases. Mutations in SERPIN family members, such as antithrombin, can cause hereditary thrombophilias. In addition, low plasma levels of SERPINs have been associated with an increased risk of thrombosis. Here, we review the biological activities of the different anticoagulant SERPINs. We further consider the clinical consequences of SERPIN deficiencies and insights gained from preclinical disease models. Finally, we discuss the potential utility of engineered SERPINs as novel therapies for the treatment of thrombotic pathologies.
影响因子：
3.7
作者：
Bhakuni T;Sharma A;Rashid Q;Kapil C;Saxena R;Mahapatra M;Jairajpuri MA
通讯作者：
Jairajpuri MA
影响因子：
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作者：
Kukkonen MK;Tiili E;Hämäläinen S;Vehmas T;Oksa P;Piirilä P;Hirvonen A
通讯作者：
Hirvonen A