期刊
FRONTIERS IN CELLULAR NEUROSCIENCE
卷 8, 期 -, 页码 -出版社
FRONTIERS MEDIA SA
DOI: 10.3389/fncel.2014.00018
关键词
Rett Syndrome; neurodevelopment; hippocampal neurons; Dendritic Spines; Dendrites; staging system; Neuronal morphology
资金
- Telethon [GGP08258]
- PRIN [2010N8PBAA]
- Compagnia San Paolo
- Fondazione Kathleen Foreman Casali-Trieste
- FRA-UniTS
- Beneficentia Stiftung-Vaduz (Lichtenstein)
Rett Syndrome (Rh) is a neurodevelopmental disorder associated with intellectual disability, mainly caused by loss-of-function mutations in the MECP2 gene. Rh brains display decreased neuronal size and dendritic arborization possibly caused by either a developmental failure or a deficit in the maintenance of dendritic arbor structure. To distinguish between these two hypotheses, the development of Mecp2-knockout mouse hippocampal neurons was analyzed in vitro. Since a staging system for the in vitro development of mouse neurons was lacking, mouse and rat hippocampal neurons development was compared between 1-15 days in vitro (DIV) leading to a 6-stage model for both species. Mecp2-knockout hippocampal neurons displayed reduced growth of dendritic branches from stage 4 (DIV4) onwards. At stages 5-6 (DIV9-15), synapse number was lowered in Mecp2-knockout neurons, suggesting increased synapse elimination. These results point to both a developmental and a maintenance setback affecting the final shape and function of neurons in RTT.
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