A study to elucidate how AdRP-associated mutations in splicing factors affect the spliceosome formation pathway

一项研究阐明剪接因子中 AdRP 相关突变如何影响剪接体形成途径

• 批准号: 21790056
• 负责人: MAITA Hiroshi
• 金额: $ 2.75万
• 依托单位: Hokkaido University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Young Scientists (B)
• 财政年份: 2009
• 资助国家: 日本
• 起止时间: 2009 至 2010
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21790056/
• 关键词: 分子生物学; pre-mRNAスプライシング; 遺伝子; シグナル伝達; スプライシング; シグナル転達

Although autosomal dominant retinitis pigmentosa (AdRP) is a tissue specific disease, disease associated mutations were found in several constitutive splicing factors consisting the spliceosome as well as genes in photo signal transduction pathway. To elucidate the mechanism how abnormality of general splicing factors triggers AdRP, we tried to detect the effect of the mutations on the cellular splicoeosme levels using a newly developed reporter. The reporter experiment suggested that the cell may regulate the spliceosome level to adjust the surrounding environment.

虽然常染色体显性视网膜色素变性（autosomal dominant retinitis pigmentosa，AdRP）是一种组织特异性疾病，但在组成剪接体的多个组成性剪接因子以及光信号转导通路的基因中发现了与疾病相关的突变。为了阐明一般剪接因子的异常如何触发AdRP的机制，我们尝试使用新开发的报告基因检测突变对细胞剪接水平的影响。报告子实验提示细胞可能通过调节剪接体水平来调节周围环境。

项目成果

Detection of free U5 snRNP level in living cells

活细胞中游离U5 snRNP水平的检测

• 发表时间: 2010
• 作者: I.Nakanishi;et al.;米田宏
• 通讯作者: 米田宏

DJ-1 binds to mitochondrial complex I and maintains its activity

• DOI: 10.1016/j.bbrc.2009.10.025
• 发表时间: 2009-12-18
• 期刊: BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
• 影响因子: 3.1
• 作者: Hayashi, Takuya;Ishimori, Chikako;Iguchi-Ariga, Sanae M. M.
• 通讯作者: Iguchi-Ariga, Sanae M. M.

Oxidative Status of DJ-1-dependent Activation of Dopamine Synthesis through Interaction of Tyrosine Hydroxylase and 4-Dihydroxy-L-phenylalanine (L-DOPA) Decarboxylase with DJ-1

• DOI: 10.1074/jbc.m109.019950
• 发表时间: 2009-10-16
• 期刊: JOURNAL OF BIOLOGICAL CHEMISTRY
• 影响因子: 4.8
• 作者: Ishikawa, Shizuma;Taira, Takahiro;Iguchi-Ariga, Sanae M. M.
• 通讯作者: Iguchi-Ariga, Sanae M. M.

Detection of free U5 snRNP levels in living cells

活细胞中游离U5 snRNP水平的检测

• 发表时间: 2010
• 作者: Maita;H.;Ariga;H.
• 通讯作者: H.

Split Luciferaseを応用した新規U5 snRNP検出法の確立

利用Split Luciferase建立新的U5 snRNP检测方法

• 发表时间: 2009
• 作者: Maita;H.;Ariga;H.
• 通讯作者: H.