ROLE OF UBIQUILIN (UBQLN) IN PROTEIN DEGENERATION

泛奎林 (UBQLN) 在蛋白质变性中的作用

• 批准号: 8360661
• 负责人: Hongmin Wang
• 金额: $ 0.25万
• 依托单位: UNIVERSITY OF SOUTH DAKOTA
• 依托单位国家: 美国
• 财政年份: 2011
• 资助国家: 美国
• 起止时间: 2011-05-01 至 2012-04-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8360661
• 关键词: Animal Model; Biochemistry; Biological Models; Biomedical Research; CAG repeat; Cell Culture Techniques; Cellular biology; Disease; Exons; Funding; Genes; Grant; Huntington Disease; Individual; Inherited; Learning; Modeling; Molecular; Molecular Genetics; National Center for Research Resources; Neuroanatomy; Neurodegenerative Disorders; Pathogenesis; Principal Investigator; Proteins; Research; Research Infrastructure; Resources; Role; Source; South Dakota; Students; United States National Institutes of Health; Work; college; cost; effective therapy; human Huntingtin protein; in vitro Model; in vivo; novel; prevent; therapeutic target; tool; ubiquilin

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. Primary support for the subproject and the subproject's principal investigator may have been provided by other sources, including other NIH sources. The Total Cost listed for the subproject likely represents the estimated amount of Center infrastructure utilized by the subproject, not direct funding provided by the NCRR grant to the subproject or subproject staff. Huntington's disease (HD) is an inherited neurodegenerative disorder caused by an abnormal expansion of CAG repeats in exon 1 of the huntingtin (Htt) gene. At present, there is no effective therapy that can either prevent HD or slow the progress of this disorder. Our research is aimed at understanding the pathogenesis of HD and identifying therapeutic targets for the treatment of this disorder. Currently, there are two undergoing directions in the lab. One is to generate and employ novel cell culture models to studying the molecular mechanisms of HD and the other is to use animal models to validate the identified potential therapeutic targets. With the in vivo and in vitro model systems, the student(s) will have an opportunity to learn and utilize the tools of neuroanatomy, cell biology, biochemistry, as well as molecular genetics to study the disorder. Specific small individual projects are available for undergraduate students, which can be completed within 10 weeks. Alexa Duling worked with Dr. Wang supported by $4000 in supplemental funds during the summer of 2010 and the lab received $1000. Dr. Wang hosted an undergraduate fellow from Mt. Marty College during the summer of 2010.

该子项目是利用资源的众多研究子项目之一 由 NIH/NCRR 资助的中心拨款提供。子项目的主要支持 并且子项目的主要研究者可能是由其他来源提供的， 包括其他 NIH 来源。 子项目可能列出的总成本 代表子项目使用的中心基础设施的估计数量， NCRR 赠款不直接向子项目或子项目工作人员提供资金。 亨廷顿病 (HD) 是一种遗传性神经退行性疾病，由亨廷顿 (Htt) 基因外显子 1 中的 CAG 重复序列异常扩增引起。目前，尚无有效的疗法可以预防 HD 或减缓这种疾病的进展。我们的研究旨在了解 HD 的发病机制并确定治疗这种疾病的治疗靶点。目前，实验室有两个正在进行的方向。一是生成并利用新型细胞培养模型来研究 HD 的分子机制，二是使用动物模型来验证已确定的潜在治疗靶点。通过体内和体外模型系统，学生将有机会学习和利用神经解剖学、细胞生物学、生物化学以及分子遗传学工具来研究该疾病。本科生可以进行特定的小型个人项目，可以在 10 周内完成。 2010 年夏天，Alexa Duling 与王博士一起工作，得到了 4000 美元的补充资金支持，实验室收到了 1000 美元。 2010 年夏天，王博士接待了来自马蒂山学院的一名本科生。