Department of Genetics, The Institute of Life Sciences, The Hebrew University of Jerusalem, Givat Ram, Jerusalem, 91904, Israel.
Adv Exp Med Biol. 2014;773:245-62. doi: 10.1007/978-1-4899-8032-8_11.
Lamins are nuclear intermediate filament proteins that are conserved in all multicellular animals. Proteins that resemble lamins are also found in unicellular organisms and in plants. Lamins form a proteinaceous meshwork that outlines the nucleoplasmic side of the inner nuclear membrane, while a small fraction of lamin molecules is also present in the nucleoplasm. They provide structural support for the nucleus and help regulate many other nuclear activities. Much of our knowledge on the function of nuclear lamins and their associated proteins comes from studies in invertebrate organisms and specifically in the nematode Caenorhabditis elegans and the fruit fly Drosophila melanogaster. The simpler lamin system and the powerful genetic tools offered by these model organisms greatly promote such studies. Here we provide an overview of recent advances in the biology of invertebrate nuclear lamins, with special emphasis on their assembly, cellular functions and as models for studying the molecular basis underlying the pathology of human heritable diseases caused by mutations in lamins A/C.
核纤层蛋白是一种存在于所有多细胞动物中的核中间丝蛋白。在单细胞生物和植物中也发现了类似于核纤层蛋白的蛋白质。核纤层蛋白形成一个蛋白网状结构,勾勒出核内膜的核质侧,而一小部分核纤层蛋白分子也存在于核质中。它们为细胞核提供结构支撑,并帮助调节许多其他核活动。我们对核纤层蛋白及其相关蛋白功能的了解主要来自于对无脊椎动物的研究,特别是在秀丽隐杆线虫和黑腹果蝇中。这些模式生物提供的简单的核纤层蛋白系统和强大的遗传工具极大地促进了这些研究。在这里,我们提供了无脊椎动物核纤层蛋白生物学的最新进展概述,特别强调了它们的组装、细胞功能以及作为研究由核纤层蛋白 A/C 突变引起的人类遗传性疾病的病理基础的分子基础的模型。