Joshi N, Kopec A K, O'Brien K M, Towery K L, Cline-Fedewa H, Williams K J, Copple B L, Flick M J, Luyendyk J P
Department of Pharmacology and Toxicology, Michigan State University, East Lansing, MI, USA; Center for Integrative Toxicology, Michigan State University, East Lansing, MI, USA.
J Thromb Haemost. 2015 Jan;13(1):57-71. doi: 10.1111/jth.12770. Epub 2014 Dec 11.
The coagulation cascade has been shown to participate in chronic liver injury and fibrosis, but the contribution of various thrombin targets, such as protease activated receptors (PARs) and fibrin(ogen), has not been fully described. Emerging evidence suggests that in some experimental settings of chronic liver injury, platelets can promote liver repair and inhibit liver fibrosis. However, the precise mechanisms linking coagulation and platelet function to hepatic tissue changes following injury remain poorly defined.
To determine the role of PAR-4, a key thrombin receptor on mouse platelets, and fibrin(ogen) engagement of the platelet αII b β3 integrin (αIIb β3 ) in a model of cholestatic liver injury and fibrosis.
Biliary and hepatic injury was characterized following 4 week administration of the bile duct toxicant α-naphthylisothiocyanate (ANIT) (0.025%) in PAR-4-deficient mice, mice expressing a mutant form of fibrin(ogen) incapable of binding integrin αII b β3 (Fibγ(Δ5) ), and wild-type mice.
Elevated plasma thrombin-antithrombin and serotonin levels, hepatic fibrin deposition, and platelet accumulation in liver accompanied hepatocellular injury and fibrosis in ANIT-treated wild-type mice. PAR-4 deficiency reduced plasma serotonin levels, increased serum bile acid concentration, and exacerbated ANIT-induced hepatocellular injury and peribiliary fibrosis. Compared with PAR-4-deficient mice, ANIT-treated Fibγ(Δ5) mice displayed more widespread hepatocellular necrosis accompanied by marked inflammation, robust fibroblast activation, and extensive liver fibrosis.
Collectively, the results indicate that PAR-4 and fibrin-αII b β3 integrin engagement, pathways coupling coagulation to platelet activation, each exert hepatoprotective effects during chronic cholestasis.
凝血级联反应已被证明参与慢性肝损伤和肝纤维化过程,但各种凝血酶靶点(如蛋白酶激活受体(PARs)和纤维蛋白(原))的作用尚未完全阐明。新出现的证据表明,在某些慢性肝损伤的实验模型中,血小板可促进肝脏修复并抑制肝纤维化。然而,损伤后凝血和血小板功能与肝组织变化之间的确切机制仍不清楚。
在胆汁淤积性肝损伤和纤维化模型中,确定小鼠血小板上关键的凝血酶受体PAR-4以及血小板αIIbβ3整合素(αIIbβ3)的纤维蛋白(原)结合对其的作用。
给PAR-4缺陷小鼠、表达不能结合整合素αIIbβ3的纤维蛋白(原)突变体(Fibγ(Δ5))的小鼠和野生型小鼠连续4周给予胆管毒物α-萘异硫氰酸酯(ANIT)(0.025%),以观察胆管和肝脏损伤情况。
在ANIT处理的野生型小鼠中,血浆凝血酶 - 抗凝血酶和血清素水平升高、肝脏纤维蛋白沉积以及肝脏中的血小板聚集与肝细胞损伤和纤维化同时出现。PAR-4缺陷降低了血浆血清素水平,增加了血清胆汁酸浓度,并加剧了ANIT诱导的肝细胞损伤和胆管周围纤维化。与PAR-4缺陷小鼠相比,ANIT处理的Fibγ(Δ5)小鼠表现出更广泛的肝细胞坏死,伴有明显炎症、强烈的成纤维细胞活化和广泛的肝纤维化。
总体而言,结果表明PAR-4和纤维蛋白-αIIbβ3整合素结合,即凝血与血小板激活的耦合途径,在慢性胆汁淤积过程中均发挥肝脏保护作用。