Hsieh C L, Donlon T A, Darras B T, Chang D D, Topper J N, Clayton D A, Francke U
Howard Hughes Medical Institute, Stanford University School of Medicine, California 94305.
Genomics. 1990 Mar;6(3):540-4. doi: 10.1016/0888-7543(90)90483-b.
Mitochondrial RNA-processing endoribonuclease (RNAase MRP) has the capacity to cleave mitochondrial RNA complementary to the light strand of the displacement loop at a unique site. The enzyme is a ribonucleoprotein whose RNA component is a nuclear gene product. The 5' flanking region of the primary transcript has control elements characteristic of RNA polymerase II transcription, and the coding region has features of RNA polymerase III transcription signals. The RNA associated with RNAase MRP is the first known RNA encoded by a single-copy gene in the nucleus and believed to be imported into mitochondria. The gene (RMRP) for this RNA component of RNAase MRP was assigned to human chromosome 9 and mouse chromosome 4 by Southern blot analyses of 11 human X rodent hybrids and 11 mouse X rodent hybrids with probe pHM1.0 and probe pSP270, respectively. In situ hybridization of probe pHSTU300 to normal human chromosomes revealed 29 of 100 cells with label on 9p and 9.6% of 302 silver grains located at 9p21--p12.
线粒体RNA加工内切核糖核酸酶（RNAase MRP）能够在一个独特位点切割与置换环轻链互补的线粒体RNA。该酶是一种核糖核蛋白，其RNA组分是一个核基因产物。初级转录本的5'侧翼区域具有RNA聚合酶II转录的控制元件特征，编码区域具有RNA聚合酶III转录信号的特征。与RNAase MRP相关的RNA是第一个已知由细胞核中单拷贝基因编码且被认为导入线粒体的RNA。通过分别用探针pHM1.0和探针pSP270对11个人类X啮齿动物杂种和11个小鼠X啮齿动物杂种进行Southern印迹分析，将RNAase MRP的这种RNA组分的基因（RMRP）定位到人类9号染色体和小鼠4号染色体。用探针pHSTU300对正常人染色体进行原位杂交显示，100个细胞中有29个在9p上有标记，302个银颗粒中有9.6%位于9p21 - p12。
