Academic Journal
Characterization of the MeCP2(R168X) Knockin Mouse Model for Rett Syndrome
| العنوان: | Characterization of the MeCP2(R168X) Knockin Mouse Model for Rett Syndrome |
|---|---|
| المؤلفون: | Wegener, Eike, Brendel, Cornelia, Fischer, Andre, Huelsmann, Swen, Gärtner, Jutta, Huppke, Peter |
| بيانات النشر: | Public Library Science |
| سنة النشر: | 2014 |
| المجموعة: | Georg-August-Universität Göttingen: GoeScholar |
| الوصف: | Rett syndrome, one of the most common causes of mental retardation in females, is caused by mutations in the X chromosomal gene MECP2. Mice deficient for MeCP2 recapitulate some of the symptoms seen in patients with Rett syndrome. It has been shown that reactivation of silent MECP2 alleles can reverse some of the symptoms in these mice. We have generated a knockin mouse model for translational research that carries the most common nonsense mutation in Rett syndrome, R168X. In this article we describe the phenotype of this mouse model. In male MeCP2(R168X) mice life span was reduced to 12-14 weeks and bodyweight was significantly lower than in wild type littermates. First symptoms including tremor, hind limb clasping and inactivity occurred at age 27 days. At age 6 weeks nest building, rotarod, open-field and elevated plus maze experiments showed impaired motor performance, reduced activity and decreased anxiety-like behavior. Plethysmography at the same time showed apneas and irregular breathing with reduced frequency. Female MeCP2R168X mice showed no significant abnormalities except decreased performance on the rotarod at age 9 months. In conclusion we show that the male MeCP2R168X mice have a phenotype similar to that seen in MECP2 knockout mouse models and are therefore well suited for translational research. The female mice, however, have a much milder and less constant phenotype making such research with this mouse model more challenging. ; Open-Access-Publikationsfonds 2015 |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | unknown |
| تدمد: | 1932-6203 |
| Relation: | https://resolver.sub.uni-goettingen.de/purl?gro-2/3379; 000347239900045; 3141995; https://resolver.sub.uni-goettingen.de/purl?gs-1/11433 |
| DOI: | 10.1371/journal.pone.0115444 |
| الاتاحة: | https://resolver.sub.uni-goettingen.de/purl?gro-2/3379 https://resolver.sub.uni-goettingen.de/purl?gs-1/11433 https://doi.org/10.1371/journal.pone.0115444 |
| Rights: | CC BY 4.0 ; https://creativecommons.org/licenses/by/4.0 |
| رقم الانضمام: | edsbas.CC86C425 |
| قاعدة البيانات: | BASE |
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