The lamin protein family

Genome Biol. 2011;12(5):222. doi: 10.1186/gb-2011-12-5-222. Epub 2011 May 31.

Abstract

The lamins are the major architectural proteins of the animal cell nucleus. Lamins line the inside of the nuclear membrane, where they provide a platform for the binding of proteins and chromatin and confer mechanical stability. They have been implicated in a wide range of nuclear functions, including higher-order genome organization, chromatin regulation, transcription, DNA replication and DNA repair. The lamins are members of the intermediate filament (IF) family of proteins, which constitute a major component of the cytoskeleton. Lamins are the only nuclear IFs and are the ancestral founders of the IF protein superfamily. Lamins polymerize into fibers forming a complex protein meshwork in vivo and, like all IF proteins, have a tripartite structure with two globular head and tail domains flanking a central α-helical rod domain, which supports the formation of higher-order polymers. Mutations in lamins cause a large number of diverse human diseases, collectively known as the laminopathies, underscoring their functional importance.

Publication types

  • Review

MeSH terms

  • Animals
  • Cardiomyopathies / genetics*
  • Cardiomyopathies / metabolism
  • Cardiomyopathies / physiopathology
  • Cell Nucleus / genetics
  • Cell Nucleus / metabolism*
  • Chromatin / metabolism
  • DNA Damage
  • DNA Repair
  • Gene Expression
  • Humans
  • Intermediate Filaments / genetics
  • Intermediate Filaments / metabolism*
  • Lamins* / classification
  • Lamins* / genetics
  • Lamins* / metabolism
  • Models, Molecular
  • Muscular Dystrophies / genetics*
  • Muscular Dystrophies / metabolism
  • Muscular Dystrophies / physiopathology
  • Mutation
  • Nuclear Envelope / genetics
  • Nuclear Envelope / metabolism*
  • Phylogeny
  • Polymerization
  • Protein Binding / genetics
  • Protein Structure, Tertiary
  • Signal Transduction

Substances

  • Chromatin
  • Lamins