Lack of Pwcr1/MBII-85 snoRNA is critical for neonatal lethality in Prader–Willi syndrome mouse models

@article{Ding2005LackOP,
  title={Lack of Pwcr1/MBII-85 snoRNA is critical for neonatal lethality in Prader–Willi syndrome mouse models},
  author={Feng Ding and Yelena Prints and Madhu Dhar and Dabney K. Johnson and Carmen Garnacho–Montero and Robert D. Nicholls and Uta Francke},
  journal={Mammalian Genome},
  year={2005},
  volume={16},
  pages={424-431}
}
Prader–Willi syndrome (PWS) is a neurobehavioral disorder caused by the lack of paternal expression of imprinted genes in the human chromosome region 15q11–13. Recent studies of rare human translocation patients narrowed the PWS critical genes to a 121-kb region containing PWCR1/HBII-85 and HBII-438 snoRNA genes. The existing mouse models of PWS that lack the expression of multiple genes, including Snrpn, Ube3a, and many intronic snoRNA genes, are characterized by 80%–100% neonatal lethality… CONTINUE READING
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