First published online August 10, 2007
Development 134, 1706e (2007)
© The Company of Biologists Limited
Segregating new development and disease roles for cohesin
The cohesin complex ensures accurate sister chromatid segregation during
cell division but it also seems to play an important role in development. For
example, mutations in several cohesin components are associated with the human
developmental disorder Cornelia de Lange syndrome (CdLS). Until now, there has
been no animal model for this syndrome but, on
p. 3191, Zhang and
co-workers report that mice lacking the cohesin regulatory protein PDS5B are
born with developmental abnormalities reminiscent of CdLS.
Pds5B-deficient mice, like people with CdLS, exhibit abnormal
skeletal patterning, heart defects and cleft palates, they report.
Unexpectedly, however, the researchers did not find any chromosome cohesion
defects in Pds5B-/- cells. Furthermore, they detected high
PDS5B expression in post-mitotic neurons of wild-type mice, identified a
DNA-binding domain in mouse PDS5B and showed that the protein localizes to the
nucleolus. Overall, these results suggest that PDS5B and the cohesin complex
might regulate multiple aspects of organogenesis by regulating developmental
gene expression rather than chromosome dynamics.
Related articles in Development:
- Mice lacking sister chromatid cohesion protein PDS5B exhibit developmental abnormalities reminiscent of Cornelia de Lange syndrome
- Bin Zhang, Sanjay Jain, Haengseok Song, Ming Fu, Robert O. Heuckeroth, Jonathan M. Erlich, Patrick Y. Jay, and Jeffrey Milbrandt
Development 2007 134: 3191-3201.
[Abstract]
[Full Text]
