DEFECTIVE TOOTH MORPHOGENESIS IN THE IFT88ORPK MUTANT MOUSE

IFT88ORPK 突变小鼠的牙齿形态发生缺陷

• 批准号: 8167770
• 负责人: Courtney Jeanne Haycraft
• 金额: $ 0.65万
• 依托单位: MEDICAL UNIVERSITY OF SOUTH CAROLINA
• 依托单位国家: 美国
• 财政年份: 2010
• 资助国家: 美国
• 起止时间: 2010-06-01 至 2011-05-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8167770
• 关键词: Bardet-Biedl Syndrome; Cilia; Computer Retrieval of Information on Scientific Projects Database; Cystic kidney; Defect; Dentition; Development; Disease; Ellis-Van Creveld Syndrome; Funding; Goals; Grant; Hydrocephalus; Institution; Mammalian Cell; Modeling; Morphogenesis; Mus; Mutant Strains Mice; Mutation; Pathology; Pattern; Play; Polydactyly; Research; Research Personnel; Resources; Role; Source; Syndrome; Tooth structure; United States National Institutes of Health; gene function; insight; kinetosome; mouse model; orofaciodigital syndrome I; skeletal

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. Primary cilia are present on most mammalian cells but their function is poorly understood in mammalian dentition. A growing number of disorders including Bardet-Biedl syndrome, Oral facial digital syndrome I, and Ellis-van Creveld syndrome have been attributed to mutations in genes that function in primary cilia or basal bodies. In addition to common pathologies such as development of renal cysts and skeletal defects, several of these disorders also have defects in tooth patterning, development or morphogenesis. Despite this evidence that primary cilia are important for the morphogenesis of the tooth, little research has been devoted to understanding the function of primary cilia in the mammalian dentition. The goal of this project is to examine the maturation of the molars in a murine model that features defective primary cilia, the Ift88orpk mutant mouse. In addition to pathologies including polydactyly, hydrocephalus and renal cysts, Ift88orpk mutant mice develop an ectopic molar. The maturation of the molars in this mouse model has not been investigated. Examination of molar morphogenesis and maturation in this mouse model will provide insight into the role primary cilia play in mammalian tooth development and how defective cilia function in these syndromes leads to changes in tooth structure.

该副本是利用众多研究子项目之一 由NIH/NCRR资助的中心赠款提供的资源。子弹和 调查员（PI）可能已经从其他NIH来源获得了主要资金， 因此可以在其他清晰的条目中代表。列出的机构是 对于中心，这不一定是调查员的机构。 原发性纤毛存在于大多数哺乳动物细胞上，但它们的功能在哺乳动物的牙列中对其作用很少。 越来越多的疾病包括Bardet-Biedl综合征，口服面部数字综合征I和Ellis-Van Creveld综合征，这归因于在原发性纤毛或基底体中起作用的基因突变。 除了常见的病理（例如肾囊肿的发育和骨骼缺陷）外，其中几种疾病还具有牙齿模式，发育或形态发生的缺陷。 尽管有证据表明原发性纤毛对于牙齿的形态发生很重要，但很少有研究致力于理解原发性纤毛在哺乳动物牙列中的功能。 该项目的目的是检查以有缺陷的原发性纤毛（IFT88ORPK突变小鼠）为具有缺陷的鼠模型中磨牙的成熟。 除了包括多肌，脑积水和肾囊肿在内的病理外，IFT88ORPK突变小鼠还会发展出异位摩尔。 该小鼠模型中磨牙的成熟尚未研究。 在该小鼠模型中检查摩尔形态发生和成熟，将洞悉原发性纤毛在哺乳动物牙齿发育中的作用，以及纤毛在这些综合征中的功能如何导致牙齿结构的变化。