Review

Chromosome Research

, Volume 21, Issue 6, pp 601-614

Long nonoding RNAs in the X-inactivation center

  • Emily MaclaryAffiliated withDepartment of Human Genetics, University of Michigan Medical School
  • , Michael HintenAffiliated withDepartment of Human Genetics, University of Michigan Medical School
  • , Clair HarrisAffiliated withDepartment of Human Genetics, University of Michigan Medical School
  • , Sundeep KalantryAffiliated withDepartment of Human Genetics, University of Michigan Medical School Email author 

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Abstract

The X-inactivation center is a hotbed of functional long noncoding RNAs in eutherian mammals. These RNAs are thought to help orchestrate the epigenetic transcriptional states of the two X-chromosomes in females as well as of the single X-chromosome in males. To balance X-linked gene expression between the sexes, females undergo transcriptional silencing of most genes on one of the two X-chromosomes in a process termed X-chromosome inactivation. While one X-chromosome is inactivated, the other X-chromosome remains active. Moreover, with a few notable exceptions, the originally established epigenetic transcriptional profiles of the two X-chromosomes is maintained as such through many rounds of cell division, essentially for the life of the organism. The stable and divergent transcriptional fates of the two X-chromosomes, despite residing in a shared nucleoplasm, make X-inactivation a paradigm of epigenetic transcriptional regulation. Originally proposed in 1961 by Mary Lyon, the X-inactivation hypothesis has been validated through much experimentation. In the last 25 years, the discovery and functional characterization has firmly established X-linked long noncoding RNAs as key players in choreographing X-chromosome inactivation.

Keywords

Xist Tsix Polycomb group Jpx Tsx Ftx RepA X-inactivation X-chromosome inactivation Histone modifications Epigenetic regulation