First published online December 7, 2008
Development 136, 13604e (2009)
© The Company of Biologists Limited
A-repeat view of X-inactivation
During early development, the inactivation of one X chromosome in female
mammals ensures equivalent X-linked gene expression in male (XY) and female
(XX) embryos. X-inactivation is triggered by the association of non-coding
Xist (X-inactive specific transcript) RNA with one X chromosome. In
ES cells, the conserved A-repeat of the Xist RNA is then required to
silence that chromosome. Now, on
p. 139, Hoki and
colleagues report that the A-repeat is also required for X-inactivation during
mouse embryogenesis. Surprisingly, however, a lack of Xist RNA,
rather than defective silencing by the mutated RNA, causes the failure of
imprinted X-inactivation (the inactivation of the paternal X in the
extraembryonic tissues) in embryos carrying a paternally transmitted
A-repeat-deleted Xist allele. Furthermore, the normally silent
paternal copy of Tsix (a negative regulator of Xist) is
ectopically activated in these A-repeat-deleted embryos. The researchers
suggest, therefore, that the genomic region encoding the A-repeat is required
for the appropriate transcriptional regulation of the Xist/Tsix loci
and subsequent X-inactivation in mouse embryos.

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Related articles in Development:
- A proximal conserved repeat in the Xist gene is essential as a genomic element for X-inactivation in mouse
- Yuko Hoki, Naomi Kimura, Minako Kanbayashi, Yuko Amakawa, Tatsuya Ohhata, Hiroyuki Sasaki, and Takashi Sado
Development 2009 136: 139-146.
[Abstract]
[Full Text]