A classification of the fibrin network structures formed from the hereditary dysfibrinogens
A classification of the fibrin network structures formed from the hereditary dysfibrinogens
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DOI：
10.1111/j.1538-7836.2006.02043.x
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发表时间：
2006-08-01
影响因子：
10.4
通讯作者：
Sakata, Y.
中科院分区：
文献类型：
--
作者：
Sugo, T.;Endo, H.;Sakata, Y.
目的：研究38种纤维蛋白原异常的分子缺陷与纤维蛋白网络的关系。方法与结果：扫描电子显微镜分析表明，在相同的条件下形成的所有纤维素的网络组成的正常厚度的纤维或薄的纤维，伴随着各种变化的网络结构和特性。我们将这些纤维蛋白网络分为五类，指定为正常、欠有序、多孔A、多孔B和花边状网络。纤维蛋白肽A释放或E：D结合位点缺陷的dysfibrinogens形成正常或较不有序的网络，而D：D结合缺陷的dysfibrinogens形成由许多锥形终止纤维组成的多孔A网络，尽管具有正常宽度的纤维，并含有许多孔或空间。多孔B和花边状网络是由高度分支的细纤维组成的，这是由于原纤维之间的横向联合缺陷，它们之间的主要区别是多孔B网络的孔隙率。所有的多孔B网络都容易被机械应力破坏，而花边状网络对这种应力保持高的抵抗力，表明网络强度不依赖于纤维宽度，而是依赖于导致网络脆性的孔隙率。结论：D：D结合障碍是导致多孔纤维蛋白网络形成的主要干扰因素。多孔性可通过D：D缔合以及侧向缔合的严重损害引入，如经常通过额外糖基化或Ca2+结合缺陷观察到的。
Objective: The main objective was to study the relationships of the molecular defects in 38 dysfibrinogens with their fibrin networks. Methods and results: Scanning electron microscopic analyses revealed that all the fibrins formed under the same conditions had networks composed of either normal thickness fibers or thin fibers, accompanied by a variety of alterations in the network structure and characteristics. We classified these fibrin networks into five classes, designated normal, less-ordered, porous A, porous B and lace-like networks. The dysfibrinogens with defects in fibrinopeptide A release or the E:D binding sites formed normal or less-ordered networks, while those with defects in the D:D association formed porous A networks composed of many tapered terminating fibers, despite having fibers of normal width, and containing many pores or spaces. The porous B and lace-like networks were composed of highly branched thin fibers because of defects in the lateral association among protofibrils, and the major difference between them was the porosity of the porous B networks. All the porous B networks were easily damaged by mechanical stress, whereas the lace-like networks retained high resistance to such stress, indicating that the network strength was not dependent on the fiber width, but on the porosity that led to fragility of the network. Conclusion: Impairment of the D:D association is the major disturbing factor that leads to the formation of porous fibrin networks. The porosity may be introduced by severe impairment of the D:D association, as well as the lateral association, as has often been observed by extra glycosylation or defects in Ca2+ binding.