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。对子项目的主要支持 子项目的首席调查员可能是由其他来源提供的， 包括美国国立卫生研究院的其他来源。为子项目列出的总成本可能 表示该子项目使用的中心基础设施的估计数量， 不是由NCRR赠款提供给次级项目或次级项目工作人员的直接资金。 亨廷顿病(HD)是一种遗传性神经退行性疾病，由亨廷顿蛋白(Htt)基因第一外显子CAG重复序列异常扩增引起。目前，还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，并确定治疗该疾病的靶点。目前，实验室中有两个正在进行的方向。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(S)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。本科生可在10周内完成具体的个人小项目。2010年夏天，Alexa Duling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。王博士招待了一位来自山上大学的本科生。马蒂学院在2010年夏天。

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.