SPINE MORPHOLOGY IN AN ANIMAL MODEL OF FRAGILE X SYNDROME

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 这些研究的主要目标是检查脆性X基因敲除小鼠模型中关键大脑区域的神经元形态，并与野生型对照进行比较。我们使用了一种融合的方法来实现这一目标，采用了传统的连续切片电子显微镜、相关荧光和光学显微镜以及三维电子显微镜等技术。这些研究的目的是为了更好地了解临床疾病--脆性X智力低下--的潜在病因和/或后果。这些研究非常有启发性，并将继续实现如下所述的目标。项目目标源于我们的发现，1)脆性X智力低下蛋白与其他蛋白质一起在突触合成，以响应代谢性谷氨酸受体的神经递质激活，以及2)功能正常的脆性X(Fmr1)基因是神经递质激活的蛋白合成所必需的。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The primary goal of these studies has been to examine the neuronal morphology in key brain areas from a mouse model of fragile X knockout mouse, compared to that of wild-type controls. We have approached this objective using a converging-methods approach, employing techniques such as conventional serial-section electron microscopy, correlative fluorescence and light microscopy, and 3-dimensional electron microscopy. These studies were designed with the intention of providing a better insight into underlying etiology and/or consequences the clinical disorder, fragile X mental retardation. These studies have been very illuminating and the objectives are continuing to be pursued as described below. Project goals arise from our findings that 1) the fragile X mental retardation protein is synthesized at synapses, along with other proteins, in response to neurotransmitter activation of metabotropic glutamate receptors, and 2) a functioning fragile X (fmr1) gene is necessary for neurotransmitter-activated protein synthesis to occur at synapses.