REGULATION OF ERAD- AND UPR-RELATED GENE EXPRESSION

ERA 和 UPR 相关基因表达的调控

• 批准号: 8170737
• 负责人: KELLEY W. MOREMEN
• 金额: $ 0.26万
• 依托单位: UNIVERSITY OF GEORGIA
• 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/8170737
• 关键词: Cells; Chemicals; Collaborations; Computer Retrieval of Information on Scientific Projects Database; Disease; Enzymes; Funding; Gene Expression; Genes; Glycoprotein Degradation Pathway; Glycoproteins; Grant; Human; Individual; Institution; Lysosomal Storage Diseases; Molecular Chaperones; Proteins; Quality Control; Regulation; Research; Research Personnel; Resources; Role; Source; Therapeutic; Transcript; United States National Institutes of Health; glycoprotein biosynthesis; response; small molecule

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. Transcript levels for various enzymes and proteins involved in human glycoprotein biosynthesis, ER-associated glycoprotein disposal (ERAD), and unfolded protein response (UPR) will be quantitated by qRT-PCR. A set of projects are being initiated in collaboration with Dr. Hung Do (Amicus Therapeutics) to examine the roles of individual genes in ER quality control and glycoprotein degradation in human cells. Amicus Therapeutics is a biotech company developing small molecule chemical chaperone therapeutics for treatment of lysosomal storage diseases. These diseases are characterized by the accumulation of misfolded lysosomal enzymes in the ER of human cells where they potentially induce an unfolded protein response.

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表示。列出的机构是 对于中心，这不一定是调查员的机构。 参与人糖蛋白生物合成的各种酶和蛋白质的转录水平，与ER相关的糖蛋白处置（ERAD）和未折叠的蛋白质反应（UPR）将通过QRT-PCR进行定量。 正在与Hung Do（Amicus Therapeatics）合作启动一组项目，以检查单个基因在ER质量控制中的作用和人类细胞中糖蛋白降解的作用。 Amicus Therapeutics是一家生物技术公司，开发了小分子化学伴侣疗法，用于治疗溶酶体储存疾病。 这些疾病的特征是在人类细胞的ER中积累了错误折叠的溶酶体酶，它们可能诱导展开的蛋白质反应。