The human keratins: biology and pathology.
The human keratins: biology and pathology.
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人角蛋白：生物学和病理学。
DOI：
10.1007/s00418-008-0435-6
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发表时间：
2008-06
影响因子：
2.3
通讯作者：
Langbein, Lutz
中科院分区：
文献类型：
--
作者：
Moll, Roland;Divo, Markus;Langbein, Lutz
角蛋白是上皮细胞典型的中间丝蛋白，呈现出高度的分子多样性。异聚体丝由I型和II型分子配对形成。人类存在54种有功能的角蛋白基因。它们以与上皮类型和细胞分化阶段高度相关的特异性模式表达。大约一半的角蛋白——包括许多近期才被鉴定的角蛋白——局限于毛囊的各个部分。作为上皮细胞骨架的一部分，角蛋白对上皮细胞和组织的机械稳定性和完整性非常重要。此外，一些角蛋白还具有调节功能，并参与细胞内信号通路，例如抗应激、伤口愈合和细胞凋亡。本文应用新的统一命名法，对所有人类角蛋白总结了它们的细胞类型和组织分布，以及与转基因小鼠模型和人类遗传性角蛋白疾病相关的功能意义。此外，由于角蛋白在人类肿瘤中也呈现出特征性的表达模式，其中一些（特别是K5、K7、K8/K18、K19和K20）在癌的免疫组织化学肿瘤诊断中非常重要，尤其是在不明转移灶以及精确分类和分型方面。未来的研究可能会为这个非凡的蛋白家族开拓更多的临床应用领域。
The keratins are the typical intermediate filament proteins of epithelia, showing an outstanding degree of molecular diversity. Heteropolymeric filaments are formed by pairing of type I and type II molecules. In humans 54 functional keratin genes exist. They are expressed in highly specific patterns related to the epithelial type and stage of cellular differentiation. About half of all keratins—including numerous keratins characterized only recently—are restricted to the various compartments of hair follicles. As part of the epithelial cytoskeleton, keratins are important for the mechanical stability and integrity of epithelial cells and tissues. Moreover, some keratins also have regulatory functions and are involved in intracellular signaling pathways, e.g. protection from stress, wound healing, and apoptosis. Applying the new consensus nomenclature, this article summarizes, for all human keratins, their cell type and tissue distribution and their functional significance in relation to transgenic mouse models and human hereditary keratin diseases. Furthermore, since keratins also exhibit characteristic expression patterns in human tumors, several of them (notably K5, K7, K8/K18, K19, and K20) have great importance in immunohistochemical tumor diagnosis of carcinomas, in particular of unclear metastases and in precise classification and subtyping. Future research might open further fields of clinical application for this remarkable protein family.
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影响因子：
7.5
作者：
Chu, PG;Wu, E;Weiss, LM
通讯作者：
Weiss, LM
影响因子：
11.4
作者：
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7.3
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7.5
作者：
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通讯作者：
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