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衰老、早衰症和核纤层蛋白病的遗传学

Genetics of aging, progeria and lamin disorders.

作者信息

Ghosh Shrestha, Zhou Zhongjun

机构信息

Shenzhen Institute of Research and Innovation, The University of Hong Kong, Shenzhen, China.

Shenzhen Institute of Research and Innovation, The University of Hong Kong, Shenzhen, China; Department of Biochemistry, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong.

出版信息

Curr Opin Genet Dev. 2014 Jun;26:41-6. doi: 10.1016/j.gde.2014.05.003. Epub 2014 Jul 6.

Abstract

Premature aging disorders, like Werner syndrome, Bloom's syndrome, and Hutchinson-Gilford Progeria Syndrome (HGPS), have been the subjects of immense interest as they recapitulate many of the phenotypes observed in physiological aging. They, therefore, not only provide model systems to study normal aging processes but also give valuable insights into the intricate mechanisms underlying senescence. Recent works on HGPS have revealed alterations in a spectrum of cellular and molecular pathways involved in the maintenance of genomic integrity, thus suggesting a profound impact of the nuclear lamina in nuclear organization, chromatin dynamics, regulation of gene expression and epigenetics.

摘要

早衰症,如沃纳综合征、布卢姆综合征和哈钦森-吉尔福德早衰综合征(HGPS),一直备受关注,因为它们重现了许多在生理性衰老中观察到的表型。因此,它们不仅为研究正常衰老过程提供了模型系统,还为衰老背后的复杂机制提供了有价值的见解。最近关于HGPS的研究揭示了一系列参与维持基因组完整性的细胞和分子途径的改变,从而表明核纤层在核组织、染色质动力学、基因表达调控和表观遗传学方面具有深远影响。

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