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核膜在层粘连蛋白病中的变化。

Changes in the Nuclear Envelope in Laminopathies.

机构信息

Biophysics & Structural Genomics Division, Saha Institute of Nuclear Physics, Kolkata, West Bengal, India.

Department of Biochemistry, University of Calcutta, Kolkata, West Bengal, India.

出版信息

Adv Exp Med Biol. 2018;1112:31-38. doi: 10.1007/978-981-13-3065-0_3.

Abstract

Double-membrane-bound nucleus is the major organelle of every metazoan cell, which controls various nuclear processes like chromatin maintenance, DNA replication, transcription and nucleoskeleton-cytoskeleton coupling. Nuclear homeostasis depends on the integrity of nuclear membrane and associated proteins. Lamins, underlying the inner nuclear membrane (INM), play a crucial role in maintaining nuclear homeostasis. In this review, we have focussed on the disruption of nuclear homeostasis due to lamin A/C mutation which produces a plethora of diseases, termed as laminopathies.

摘要

双层膜包被的细胞核是所有后生动物细胞的主要细胞器,它控制着各种核过程,如染色质维持、DNA 复制、转录和核骨架-细胞骨架偶联。核内环境稳定依赖于核膜及其相关蛋白的完整性。位于核内膜(INM)之下的核纤层蛋白在维持核内环境稳定中起着至关重要的作用。在这篇综述中,我们重点讨论了由于核纤层蛋白 A/C 突变导致的核内环境稳定破坏,这种突变产生了多种疾病,称为核纤层病。

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