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中间纤维(intermediate filament,IF)与微管、微丝一起组成细胞骨架的蛋白质纤维网络体系。三种骨架纤维中最复杂的是IF,它由最大的基因家族所编码,组成了一个包含73个成员的蛋白大家族。IF除了支架作用还形成复杂的信息平台,并与各种激酶、受体和凋亡蛋白相互作用。目前,已知80多种人类相关疾病包括皮肤起泡、肌肉萎缩症、心肌病、早衰综合征、神经退行性疾病和白内障等与IF有关,且数量仍在增长。其中,IF的变异至少与30多种人类组织特异性疾病有关,在几种神经退行性疾病、肌肉疾病或其他相关疾病还会出现特征性的包涵体。IF可作为细胞类型的标志,其抗体被广泛应用于病理诊断,因此研究这些疾病与IF之间的相互联系、揭示它们的作用机制对全面认识IF在细胞和组织中所起的作用以及对临床疾病的治疗有着重要意义。
The middle fiber is a protein network with cytoskeleton formed by microtubules and microfilaments. The most complex of the three skeletal fibers is IF, which is encoded by the largest gene family and comprises a large family of 73 proteins. In addition to the scaffold effect, IF also forms a complex information platform and interacts with various kinases, receptors and apoptotic proteins. Currently, it is known that more than 80 kinds of human-related diseases, such as skin foaming, muscular dystrophy, cardiomyopathy, premature aging syndrome, neurodegenerative diseases and cataracts, are related to IF and are still in increasing numbers. Among them, the variation of IF is associated with at least 30 kinds of human tissue-specific diseases, and characteristic inclusion bodies appear in several neurodegenerative diseases, muscle diseases or other related diseases. IF can be used as a marker of cell type. Antibodies are widely used in pathological diagnosis. Therefore, to study the relationship between these diseases and IF, reveal their mechanism of action to fully understand the role of IF in cells and tissues and to clinical The treatment of diseases is of great importance.