SPINE MORPHOLOGY IN AN ANIMAL MODEL OF FRAGILE X SYNDROME

脆性 X 综合征动物模型的脊柱形态

• 批准号: 7601659
• 负责人: WILLIAM T GREENOUGH
• 金额: $ 0.18万
• 依托单位: UNIVERSITY OF CALIFORNIA, SAN DIEGO
• 依托单位国家: 美国
• 财政年份: 2007
• 资助国家: 美国
• 起止时间: 2007-05-01 至 2008-04-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7601659
• 关键词: Actins; Animal Model; Appearance; Autopsy; Computer Retrieval of Information on Scientific Projects Database; Dendrites; Dendritic Spines; Disease; F-Actin; FMRP; Fragile X Mental Retardation Protein; Fragile X Syndrome; Funding; Golgi Apparatus; Grant; Human; Institution; Link; Mental Retardation; Methodology; Morphology; Research; Research Personnel; Resources; Shapes; Source; Staining method; Stains; Synapses; Three-Dimensional Imaging; Tissues; United States National Institutes of Health; Vertebral column; mouse model; voltage

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. Fragile X mental retardation, a genetically-linked disorder, results from the inability to produce the protein FMRP. The lack of FMRP expression has been associated with altered dendritic spine morphology in human post-mortem tissue and a mouse model of the disorder. Moreover, stability of spine shape has been shown to be dependent upon the filamentous form of actin, F-actin. Golgi-impregnation studies of human autopsy tissue and of a mouse model of fragile X mental retardation syndrome suggest that dendritic spines have an underdeveloped appearance--a tendency towards elongated, tortuous spine morphology. We have tentatively hypothesized that the absence of the fragile X protein (FMRP) interferes with synaptic and dendritic maturation. With the NCMIR this we will examine dendritic segments using high voltage EM and NCMIR's recently developed actin staining methodology to provide 3-D images of dendrites and their spines to evaluate and provide more details with respect to the maturation hypothesis.

该子项目是利用该技术的众多研究子项目之一 资源由 NIH/NCRR 资助的中心拨款提供。子项目和 研究者 (PI) 可能已从 NIH 的另一个来源获得主要资金， 因此可以在其他 CRISP 条目中表示。列出的机构是 对于中心来说，它不一定是研究者的机构。 脆性 X 型智力低下是一种与遗传相关的疾病，是由于无法产生 FMRP 蛋白造成的。 FMRP 表达的缺乏与人类死后组织和小鼠模型中树突棘形态的改变有关。 此外，脊柱形状的稳定性已被证明取决于肌动蛋白（F-肌动蛋白）的丝状形式。 对人体尸检组织和脆性 X 智力低下综合征小鼠模型的高尔基体浸渍研究表明，树突棘具有不发达的外观，即有拉长、曲折的脊柱形态倾向。 我们初步假设脆性 X 蛋白 (FMRP) 的缺失会干扰突触和树突的成熟。 借助 NCMIR，我们将使用高压 EM 和 NCMIR 最近开发的肌动蛋白染色方法来检查树突片段，以提供树突及其脊柱的 3D 图像，以评估并提供有关成熟假设的更多详细信息。