Nuclear Lamins: Thin Filaments with Major Functions

Trends Cell Biol. 2018 Jan;28(1):34-45. doi: 10.1016/j.tcb.2017.08.004. Epub 2017 Sep 8.

Abstract

The nuclear lamina is a nuclear peripheral meshwork that is mainly composed of nuclear lamins, although a small fraction of lamins also localizes throughout the nucleoplasm. Lamins are classified as type V intermediate filament (IF) proteins. Mutations in lamin genes cause at least 15 distinct human diseases, collectively termed laminopathies, including muscle, metabolic, and neuronal diseases, and can cause accelerated aging. Most of these mutations are in the LMNA gene encoding A-type lamins. A growing number of nuclear proteins are known to bind lamins and are implicated in both nuclear and cytoskeletal organization, mechanical stability, chromatin organization, signaling, gene regulation, genome stability, and cell differentiation. Recent studies reveal the organization of the lamin filament meshwork in somatic cells where they assemble as tetramers in cross-section of the filaments.

Keywords: cryo-electron tomography; lamin-binding proteins; laminopathies; nuclear envelope; nuclear lamina.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Chromatin / metabolism
  • Cryoelectron Microscopy
  • Gene Expression Regulation*
  • Humans
  • Intermediate Filaments / metabolism
  • Lamins / genetics
  • Lamins / metabolism*
  • Models, Molecular
  • Mutation
  • Nuclear Lamina / genetics
  • Nuclear Lamina / metabolism*
  • Nuclear Lamina / ultrastructure
  • Nuclear Pore / metabolism

Substances

  • Chromatin
  • Lamins