线粒体 tRNA 修饰
中文名称
通路描述
线粒体基因组编码的 22 种 tRNA 在由核编码并导入线粒体的酶在线粒体基质中修饰（综述 Suzuki 等 2011, Salinas-Giege 等 2015）。例如 PUS1 和 TRIT1 等酶位于多个隔室，修饰线粒体 tRNA 和胞质 tRNA。其他酶如 MTO1、TRMU 和 TRMT61B 仅存在于线粒体中。位于 tRNA 反密码子和 3' 端的修饰通常影响 tRNA 与 mRNA 在核糖体中的相互作用以及与 tRNA 合成酶的相互作用，而其他区域的修饰（通常在 tRNA 的“核心”区域）则影响 tRNA 的折叠和稳定性（综述 Hou 等 2015）。至少 3 种 tRNA 的反密码子中存在的异常修饰 5-硫代牛磺基 -2-硫尿苷 -34 仅存在于哺乳动物线粒体中，影响负责生物合成酶的突变（GTPBP3、MTO1、TRMU）会导致线粒体功能障碍（综述 Torres 等 2014）。
英文描述
tRNA modification in the mitochondrion The 22 tRNAs encoded by the mitochondrial genome are modified in the mitochondrial matrix by enzymes encoded in the nucleus and imported into mitochondria (reviewed in Suzuki et al. 2011, Salinas-Giege et al. 2015). Some enzymes such as PUS1 and TRIT1 are located in more than one compartment and modify both mitochondrial tRNAs and cytosolic tRNAs. Other enzymes such as MTO1, TRMU, and TRMT61B are exclusively mitochondrial.
Modifications near the anticodon and near the 3' end of tRNAs tend to affect interaction of the tRNA with mRNA within ribosomes and with tRNA synthetases, respectively. Modifications in other regions, typically in the "core" of the tRNA tend to affect folding and stability of the tRNA (reviewed in Hou et al. 2015). The unusual modification 5-taurinomethyl-2-thiouridine-34 in the anticodon of at least 3 tRNAs is found only in mammalian mitochondria and mutations that affect the responsible biosynthetic enzymes (GTPBP3, MTO1, TRMU) cause mitochondrial dysfunction and disease (reviewed in Torres et al. 2014).
Modifications near the anticodon and near the 3' end of tRNAs tend to affect interaction of the tRNA with mRNA within ribosomes and with tRNA synthetases, respectively. Modifications in other regions, typically in the "core" of the tRNA tend to affect folding and stability of the tRNA (reviewed in Hou et al. 2015). The unusual modification 5-taurinomethyl-2-thiouridine-34 in the anticodon of at least 3 tRNAs is found only in mammalian mitochondria and mutations that affect the responsible biosynthetic enzymes (GTPBP3, MTO1, TRMU) cause mitochondrial dysfunction and disease (reviewed in Torres et al. 2014).
所含基因
10 个基因