Mutant mtDNA at 1555 A to G in 12S rRNA gene and hypersusceptibility of mitochondrial translation to streptomycin can be co-transferred to rho 0 HeLa cells.
Mutant mtDNA at 1555 A to G in 12S rRNA gene and hypersusceptibility of mitochondrial translation to streptomycin can be co-transferred to rho 0 HeLa cells.
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12S rRNA 基因中 1555 A 至 G 处的突变 mtDNA 和线粒体翻译对链霉素的高度敏感性可共同转移至 rho 0 HeLa 细胞。
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发表时间：
1996
期刊：
影响因子：
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通讯作者：
J. Hayashi
中科院分区：
文献类型：
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作者：
K. Inoue;D. Takai;A. Soejima;K. Isobe;Tatsuya Yamasoba;Yoshitomo Oka;Yu;J. Hayashi
从日本氨基糖苷性耳聋患者母亲分离的人皮肤成纤维细胞系95-119，线粒体DNA (mtDNA) 12S rRNA基因1555 a转G突变，研究1555 mtDNA突变与其致病性的关系。通过将携带或不携带1555突变的mtDNA转移到mtDNA缺失（rho 0）的HeLa细胞中，我们分离出了细胞系克隆，发现携带1555同源突变的细胞系克隆的线粒体翻译对链霉素的敏感性最高。这些观察结果表明，在95-119成纤维细胞中观察到的突变mtDNA的基因型和对链霉素敏感的表型同时共转移到rho 0 HeLa细胞，支持了同源1555 mtDNA突变参与导致氨基糖苷性听力损失的发病机制的观点。
Human skin fibroblast line 95-119, which had been isolated from the mother of a Japanese patient with aminoglycoside-induced deafness and a 1555 A to G mutation at 12S rRNA gene in mitochondrial DNA (mtDNA), was used to investigate the relationship between the 1555 mtDNA mutation and its pathogenicity. By the intercellular transfer of mtDNA with or without the 1555 mutation to mtDNA-less (rho 0) HeLa cells, we isolated cybrid clones and found that the mitochondrial translation in a cybrid clone repopulated with the homoplasmic 1555 mutation showed the highest susceptibility to streptomycin. These observations suggest that the genotype of the mutant mtDNA and the phenotype of hypersusceptibility to streptomycin observed in 95-119 fibroblasts were co-transferred simultaneously to rho 0 HeLa cells, supporting the idea that the homoplasmic 1555 mtDNA mutation is involved in the pathogenesis leading to aminoglycoside-induced hearing loss.