The nuclear envelopathies and human diseases

J Biomed Sci. 2009 Oct 22;16(1):96. doi: 10.1186/1423-0127-16-96.

Abstract

The nuclear envelope (NE) consists of two membrane layers that segregate the nuclear from the cytoplasmic contents. Recent progress in our understanding of nuclear-lamina associated diseases has revealed intriguing connections between the envelope components and nuclear processes. Here, we review the functions of the nuclear envelope in chromosome organization, gene expression, DNA repair and cell cycle progression, and correlate deficiencies in envelope function with human pathologies.

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cell Cycle
  • Chromosomes / metabolism
  • Cytoplasm / metabolism
  • DNA Repair
  • Gene Expression Profiling
  • Gene Expression Regulation
  • Humans
  • Lamin Type A / metabolism
  • Lamin Type B / metabolism
  • Mice
  • Models, Biological
  • Nuclear Envelope / metabolism*
  • Nuclear Lamina / metabolism*
  • Nuclear Pore Complex Proteins / metabolism

Substances

  • Lamin Type A
  • Lamin Type B
  • Nuclear Pore Complex Proteins
  • lamin B1