Centre for Cellular and Molecular Biology (CSIR), Hyderabad 500 007, India.
J Biosci. 2011 Aug;36(3):471-9. doi: 10.1007/s12038-011-9085-2.
Lamins are major structural proteins of the nucleus and are essential for nuclear integrity and organization of nuclear functions. Mutations in the human lamin genes lead to highly degenerative genetic diseases that affect a number of different tissues such as muscle, adipose or neuronal tissues, or cause premature ageing syndromes. New findings on the role of lamins in cellular signalling pathways, as well as in ubiquitin-mediated proteasomal degradation, have given important insights into possible mechanisms of pathogenesis.
核纤层蛋白是细胞核的主要结构蛋白,对于核的完整性和核功能的组织至关重要。人类核纤层蛋白基因突变可导致多种组织(如肌肉、脂肪或神经组织)发生退行性遗传疾病,或引起早衰综合征。核纤层蛋白在细胞信号通路以及泛素介导的蛋白酶体降解中的作用的新发现,为发病机制的可能机制提供了重要的见解。