作者
Daniela Tropea, Emanuela Giacometti, Nathan R Wilson, Caroline Beard, Cortina McCurry, Dong Dong Fu, Ruth Flannery, Rudolf Jaenisch, Mriganka Sur
发表日期
2009/2/10
期刊
Proceedings of the National Academy of Sciences
卷号
106
期号
6
页码范围
2029-2034
出版商
National Academy of Sciences
简介
Rett Syndrome (RTT) is a severe form of X-linked mental retardation caused by mutations in the gene coding for methyl CpG-binding protein 2 (MECP2). Mice deficient in MeCP2 have a range of physiological and neurological abnormalities that mimic the human syndrome. Here we show that systemic treatment of MeCP2 mutant mice with an active peptide fragment of Insulin-like Growth Factor 1 (IGF-1) extends the life span of the mice, improves locomotor function, ameliorates breathing patterns, and reduces irregularity in heart rate. In addition, treatment with IGF-1 peptide increases brain weight of the mutant mice. Multiple measurements support the hypothesis that RTT results from a deficit in synaptic maturation in the brain: MeCP2 mutant mice have sparse dendritic spines and reduced PSD-95 in motor cortex pyramidal neurons, reduced synaptic amplitude in the same neurons, and protracted cortical plasticity …
引用总数
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D Tropea, E Giacometti, NR Wilson, C Beard… - Proceedings of the National Academy of Sciences, 2009