Diverse lamin-dependent mechanisms interact to control chromatin dynamics. Focus on laminopathies

Daria Camozzi, Cristina Capanni, Vittoria Cenni, Elisabetta Mattioli, Marta Columbaro, Stefano Squarzoni, Giovanna Lattanzi

Research output: Contribution to journalArticle

Abstract

Interconnected functional strategies govern chromatin dynamics in eukaryotic cells. In this context, A and B type lamins, the nuclear intermediate filaments, act on diverse platforms involved in tissue homeostasis. On the nuclear side, lamins elicit large scale or fine chromatin conformational changes, affect DNA damage response factors and transcription factor shuttling. On the cytoplasmic side, bridging-molecules, the LINC complex, associate with lamins to coordinate chromatin dynamics with cytoskeleton and extra-cellular signals.   Consistent with such a fine tuning, lamin mutations and/or defects in their expression or post-translational processing, as well as mutations in lamin partner genes, cause a heterogeneous group of diseases known as laminopathies. They include muscular dystrophies, cardiomyopathy, lipodystrophies, neuropathies, and progeroid syndromes. The study of chromatin dynamics under pathological conditions, which is summarized in this review, is shedding light on the complex and fascinating role of the nuclear lamina in chromatin regulation.

Original languageEnglish
Pages (from-to)427-440
Number of pages14
JournalNucleus (Austin, Tex.)
Volume5
Issue number5
DOIs
Publication statusPublished - Sep 1 2014

Keywords

  • chromatin
  • Emery-Dreifuss muscular dystrophy
  • familial partial lipodystrophy
  • Hutchinson-Gilford progeria
  • LADs
  • laminopathies
  • lamins
  • mandibuloacral dysplasia
  • nuclear envelope proteins

ASJC Scopus subject areas

  • Medicine(all)

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