Toivola Diana M, Boor Peter, Alam Catharina, Strnad Pavel
Department of Biosciences, Cell Biology, Åbo Akademi University and Turku Center for Disease Modeling, University of Turku, Turku, Finland.
Institute of Pathology and Department of Nephrology, RWTH University, Aachen, Germany; Institute of Molecular Biomedicine, Comenius University, Bratislava, Slovakia.
Curr Opin Cell Biol. 2015 Feb;32:73-81. doi: 10.1016/j.ceb.2014.12.008. Epub 2015 Jan 17.
The cytoprotective keratins (K) compose the intermediate filaments of epithelial cells and their inherited and spontaneous mutations give rise to keratinopathies. For example, mutations in K1/K5/K10/K14 cause epidermal skin diseases whereas simple epithelial K8/K18/K19 variants predispose to development of several liver disorders. Due to their abundance, tissue- and context-specific expression, keratins constitute excellent diagnostic markers of both neoplastic and non-neoplastic diseases. During injury and in disease, keratin expression levels, cellular localization or posttranslational modifications are altered. Accumulating evidence suggests that these changes modulate multiple processes including cell migration, tumor growth/metastasis and development of infections. Therefore, our understanding of keratins is shifting from diagnostic markers to active disease modifiers.
细胞保护角蛋白（K）构成上皮细胞的中间丝，其遗传性和自发性突变会引发角蛋白病。例如，K1/K5/K10/K14的突变会导致表皮性皮肤病，而简单上皮的K8/K18/K19变体则易引发多种肝脏疾病。由于角蛋白含量丰富、具有组织和环境特异性表达，它们是肿瘤性和非肿瘤性疾病的优秀诊断标志物。在损伤和疾病过程中，角蛋白的表达水平、细胞定位或翻译后修饰会发生改变。越来越多的证据表明，这些变化会调节多个过程，包括细胞迁移、肿瘤生长/转移和感染的发展。因此，我们对角蛋白的认识正从诊断标志物转向活跃的疾病调节因子。
