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Publication : Neocortical vasculature abnormalities in the Fragile X mental retardation syndrome.

First Author  Galvan AM Year  2012
Journal  Brain Res Volume  1471
Pages  155-61 PubMed ID  22796598
Mgi Jnum  J:192392 Mgi Id  MGI:5465036
Doi  10.1016/j.brainres.2012.06.056 Citation  Galvan AM, et al. (2012) Neocortical vasculature abnormalities in the Fragile X mental retardation syndrome. Brain Res 1471:155-61
abstractText  The Fragile X syndrome (FXS) is the leading form of inherited mental retardation. To date, the most prominent neuronal phenotype associated with the syndrome is an abundance of long thin spines exhibiting an immature morphology. However, in addition to synaptic abnormalities, recent case studies have demonstrated that Fragile X (FX) patients also exhibit abnormal cerebral blood flow (CBF). To examine the role of the Fragile X mental retardation protein (FMRP) in altering CBF, we examined blood vessel density (BVD) in the visual cortex of Adult and Middle-aged FX mice. Analysis of Middle-aged FX mice demonstrated elevated BVD compared to wildtype controls, suggesting that FX mice exhibit a lack of age-induced BVD plasticity. However, Adult FX and wildtype mice did not exhibit consistent differences in BVD. These data demonstrate that FMRP is required for age-induced neocortical vasculature plasticity. Furthermore, these data suggest a new role for FMRP in blood vessel regulation that would have profound implications towards appropriately timed delivery of neuronal nutrients, thus contributing to or exacerbating FX cognitive and neuronal abnormalities.
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