Brain Lipid Analysis in Mice with Rett Syndrome |
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Authors: | Thomas N Seyfried Karie A Heinecke John G Mantis Christine A Denny |
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Institution: | (1) Department of Biology, Boston College, Chestnut Hill, Boston, MA 02467, USA;(2) Present address: Department of Biological Sciences, Columbia University, 1051 Riverside Drive, Columbia, NY 10032, USA |
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Abstract: | Rett syndrome (RS) is an X-linked neurodevelopmental disorder mostly involving mutations in the gene for methyl-CpG-binding
protein 2 (MECP2). Ganglioside abnormalities were previously found in cerebrum and cerebellum in RS patients. We evaluated total lipid distribution
in cerebrum/brainstem, hippocampus, and cerebellum in male mice carrying either the Mecp2
tm1.1Bird knockout mutation or the Mecp2
308/y deletion mutation. The concentration of the neuronal enriched ganglioside GD1a was significantly lower in the cerebrum/brainstem
of Mecp2
tm1.1Bird mice than in that of age matched controls, but was not reduced in the Mecp2
308/y mice. No other differences in brain lipid content, including myelin-enriched cerebrosides, were detected in mice with either
type of Mecp2 mutation. These findings indicate that the poor motor performance previously reported in the RS mutant mice is not associated
with major brain lipid abnormalities and that most previous brain lipid abnormalities observed in RS patients were not observed
in the Mecp2
tm1.1Bird or the Mecp2
308/y RS mice. |
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Keywords: | Mecp2 Rett Mouse models Gangliosides GD1a Myelin |
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