Elg1, a central player in genome stability

Mutat Res Rev Mutat Res. 2015 Jan-Mar:763:267-79. doi: 10.1016/j.mrrev.2014.11.007. Epub 2014 Nov 24.


ELG1 is a conserved gene uncovered in a number of genetic screens in yeast aimed at identifying factors important in the maintenance of genome stability. Elg1's activity prevents gross chromosomal rearrangements, maintains proper telomere length regulation, helps repairing DNA damage created by a number of genotoxins and participates in sister chromatid cohesion. Elg1 is evolutionarily conserved, and its mammalian ortholog (also known as ATAD5) is embryonic lethal when lost in mice, acts as a tumor suppressor in mice and humans, exhibits physical interactions with components of the human Fanconi Anemia pathway and may be responsible for some of the phenotypes associated with neurofibromatosis. In this review, we summarize the information available on Elg1-related activities in yeast and mammals, and present models to explain how the different phenotypes observed in the absence of Elg1 activity are related.

Keywords: Chromatin; DNA repair; DNA replication; Genome stability; Sister chromatid cohesion; Telomere length regulation.

Publication types

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

MeSH terms

  • ATPases Associated with Diverse Cellular Activities
  • Adenosine Triphosphatases / genetics
  • Adenosine Triphosphatases / metabolism*
  • Animals
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism*
  • Fanconi Anemia Complementation Group Proteins / metabolism*
  • Fungal Proteins / metabolism
  • Genome
  • Genomic Instability*
  • Humans
  • Mice
  • Models, Genetic
  • Neurofibromatoses / metabolism
  • Neurofibromatoses / pathology
  • Saccharomycetales / metabolism


  • ATAD5 protein, human
  • DNA-Binding Proteins
  • Fanconi Anemia Complementation Group Proteins
  • Fungal Proteins
  • ATAD5 protein, mouse
  • Adenosine Triphosphatases
  • ATPases Associated with Diverse Cellular Activities