Changes in the Nuclear Envelope in Laminopathies

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.

Keywords: Emerin; Lamin B receptor; Laminopathies; Lamins; Linker of nucleoskeleton and cytoskeleton; Nuclear pore complex.

Publication types

  • Review

MeSH terms

  • Cell Nucleus
  • Cytoskeleton
  • Homeostasis
  • Humans
  • Lamin Type A / genetics*
  • Mutation*
  • Nuclear Envelope / pathology*

Substances

  • LMNA protein, human
  • Lamin Type A
  • lamin C