Weisel John W, Litvinov Rustem I
Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
Subcell Biochem. 2017;82:405-456. doi: 10.1007/978-3-319-49674-0_13.
Fibrinogen and fibrin are essential for hemostasis and are major factors in thrombosis, wound healing, and several other biological functions and pathological conditions. The X-ray crystallographic structure of major parts of fibrin(ogen), together with computational reconstructions of missing portions and numerous biochemical and biophysical studies, have provided a wealth of data to interpret molecular mechanisms of fibrin formation, its organization, and properties. On cleavage of fibrinopeptides by thrombin, fibrinogen is converted to fibrin monomers, which interact via knobs exposed by fibrinopeptide removal in the central region, with holes always exposed at the ends of the molecules. The resulting half-staggered, double-stranded oligomers lengthen into protofibrils, which aggregate laterally to make fibers, which then branch to yield a three-dimensional network. Much is now known about the structural origins of clot mechanical properties, including changes in fiber orientation, stretching and buckling, and forced unfolding of molecular domains. Studies of congenital fibrinogen variants and post-translational modifications have increased our understanding of the structure and functions of fibrin(ogen). The fibrinolytic system, with the zymogen plasminogen binding to fibrin together with tissue-type plasminogen activator to promote activation to the active proteolytic enzyme, plasmin, results in digestion of fibrin at specific lysine residues. In spite of a great increase in our knowledge of all these interconnected processes, much about the molecular mechanisms of the biological functions of fibrin(ogen) remains unknown, including some basic aspects of clotting, fibrinolysis, and molecular origins of fibrin mechanical properties. Even less is known concerning more complex (patho)physiological implications of fibrinogen and fibrin.
纤维蛋白原和纤维蛋白对于止血至关重要,并且是血栓形成、伤口愈合以及其他几种生物学功能和病理状况的主要因素。纤维蛋白原主要部分的X射线晶体结构,连同缺失部分的计算重建以及大量的生化和生物物理研究,提供了丰富的数据来解释纤维蛋白形成、其组织和特性的分子机制。凝血酶切割纤维蛋白肽后,纤维蛋白原转化为纤维蛋白单体,这些单体通过中央区域纤维蛋白肽去除后暴露的“旋钮”相互作用,而分子末端始终暴露有“孔洞”。由此产生的半交错双链寡聚体延伸成长原纤维,原纤维横向聚集形成纤维,然后分支形成三维网络。现在我们对凝块机械性能的结构起源已经有了很多了解,包括纤维取向、拉伸和屈曲的变化以及分子结构域的强制展开。对先天性纤维蛋白原变体和翻译后修饰的研究增进了我们对纤维蛋白原结构和功能的理解。纤维蛋白溶解系统中,酶原纤溶酶原与组织型纤溶酶原激活剂结合在纤维蛋白上,促进激活为活性蛋白水解酶纤溶酶,导致纤维蛋白在特定赖氨酸残基处被消化。尽管我们对所有这些相互关联的过程的了解有了很大增加,但关于纤维蛋白原生物学功能的分子机制仍有很多未知之处,包括凝血、纤维蛋白溶解的一些基本方面以及纤维蛋白机械性能的分子起源。关于纤维蛋白原和纤维蛋白更复杂的(病理)生理意义,我们了解得更少。