ROLE OF UBIQUILIN (UBQLN) IN PROTEIN DEGENERATION

这个子项目是利用资源的许多研究子项目之一 由NIH/NCRR资助的中心拨款提供。次级项目的主要支助 子项目的主要研究者可能是由其他来源提供的， 包括其它NIH来源。 列出的子项目总成本可能 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）是由亨廷顿蛋白（Htt）基因外显子1中CAG重复序列异常扩增引起的遗传性神经退行性疾病。目前，没有有效的治疗方法可以预防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，实验室正在进行两个方向的研究。一是建立和应用新的细胞培养模型来研究HD的分子机制，二是利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的工具来研究这种疾病。具体的小的个人项目可供本科生，这可以在10周内完成。 Alexa Duling在2010年夏天与王博士合作，获得了4000美元的补充资金，实验室获得了1000美元。 王博士接待了一位来自中山大学的本科生。2010年夏天，马蒂学院。

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.