Department of Genetics, Institute of Life Sciences, Hebrew University of Jerusalem, Jerusalem 91904, Israel.
EMBO Rep. 2012 Dec;13(12):1070-8. doi: 10.1038/embor.2012.167. Epub 2012 Nov 13.
Lamins are nuclear intermediate filament proteins. They provide mechanical stability, organize chromatin and regulate transcription, replication, nuclear assembly and nuclear positioning. Recent studies provide new insights into the role of lamins in development, differentiation and tissue response to mechanical, reactive oxygen species and thermal stresses. These studies also propose the existence of separate filament networks for A- and B-type lamins and identify new roles for the different networks. Furthermore, they show changes in lamin composition in different cell types, propose explanations for the more than 14 distinct human diseases caused by lamin A and lamin C mutations and propose a role for lamin B1 in these diseases.
核纤层蛋白是核内中间丝蛋白。它们提供机械稳定性,组织染色质并调节转录、复制、核组装和核定位。最近的研究为核纤层蛋白在发育、分化以及组织对机械、活性氧和热应激的反应中的作用提供了新的认识。这些研究还提出了 A 型和 B 型核纤层蛋白存在独立的丝网络,并确定了不同网络的新作用。此外,它们显示了不同细胞类型中核纤层蛋白组成的变化,为 14 种以上由核纤层蛋白 A 和核纤层蛋白 C 突变引起的不同人类疾病提出了解释,并提出核纤层蛋白 B1 在这些疾病中的作用。