PWS-IC mice

Model

Jackson Laboratory (Sasner, Michael)

Bioinformatics and Model Development

The Jackson Laboratory

Michael Sasner (msasner)

Description

When females heterozygous for the PWS-ICdel mutation are bred with wildtype males, the resulting offspring are viable and fertile as adults. In contrast, paternal transmission of the PWS-ICdel mutation results in neonatal lethality between 1-7 days of age (failure to thrive) due to imprinting defects. Most of these pups will die within 48 hours, however, a few live to 7 days. Pups are underweight, unable to support themselves, dehydrated and weak. Although capable of nursing, little milk is observed in their stomachs and they develop low blood glucose levels. Of note, removing all but one wildtype littermate improves mutant mouse survivability. In addition, mutant mouse viability may be greatly improved by outcrossing to FVB/NJ females. These PWS-ICdel mutant mice may be useful in studying the Prader Willi syndrome imprinting center.

Yang T; Adamson TE; Resnick JL; Leff S; Wevrick R; Francke U ; Jenkins NA ; Copeland NG ; Brannan CI. 1998. A mouse model for Prader-Willi syndrome imprinting-centre mutations. Nat Genet 19(1):25-31. [PubMed: 9590284]

B6.129-Snrpntm2Cbr/J

For more information see:  http://jaxmice.jax.org/strain/012443.html 

Created on: August 6, 2013 @ 1:16pm

Last Modified on: August 6, 2013 @ 1:23pm