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Publication : Longitudinal brain MRI study in a mouse model of Rett Syndrome and the effects of choline.

First Author  Ward BC Year  2008
Journal  Neurobiol Dis Volume  31
Issue  1 Pages  110-9
PubMed ID  18571096 Mgi Jnum  J:139191
Mgi Id  MGI:3807442 Doi  10.1016/j.nbd.2008.03.009
Citation  Ward BC, et al. (2008) Longitudinal brain MRI study in a mouse model of Rett Syndrome and the effects of choline. Neurobiol Dis 31(1):110-9
abstractText  Rett Syndrome (RTT), the second most common cause of mental retardation in girls, is associated with mutations of an X-linked gene encoding the transcriptional repressor protein MeCP2. Mecp2(1lox) mutant mice express no functional MeCP2 protein and exhibit behavioral abnormalities similar to those seen in RTT patients. Here we monitor the development of both whole brain and regional volumes between 21 and 42 days of age in this model of RTT using MRI. We see decreases in whole brain volumes in both male and female mutant mice. Cerebellar and ventricular volumes are also decreased in RTT males. Previous work has suggested that perinatal choline supplementation alleviates some of the behavioral deficits in both male and female Mecp2(1lox) mutant mice. Here we show that perinatal choline supplementation also positively affects whole brain volume in heterozygous females, and cerebellar volume in male RTT mice.
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