Inter-mitochondrial complementation of mtDNA mutations and nuclear context
Inter-mitochondrial complementation of mtDNA mutations and nuclear context
复制标题
mtDNA 突变和核背景的线粒体间互补
作者：
G. Attardi;J. Enríquez;J. Cabezas
Ono等人报道了在细胞杂交中，两种类型的线粒体之间存在广泛的线粒体间互补（反式互补），这两种类型的线粒体在tRNA^（Ile）和tRNA^（Leu（UUR））基因中携带两种人类mtDNA致病性隐性突变之一。几乎所有的杂种在融合后10至14天表现出正常的线粒体蛋白质合成和呼吸活性，但不是更早。他们的结果与我们之前的观察形成鲜明对比，后者清楚地表明，同一人类细胞中不同细胞器内携带的两种不同有害线粒体DNA突变之间确实可能发生互补，但在所分析的细胞中这种现象很少见。我们希望指出，两个实验室结论之间的差异并不涉及哺乳动物线粒体是否具有在体内融合和混合其mtDNA和/或mtDNA产物的潜力的问题，我们对这个问题并不持异议，而是与这种现象的普遍性和频率以及由细胞核控制的重要性有关。
Ono et al. report extensive inter- mitochondrial complementation (transcomplementation) in cell hybrids between two types of respiratory-deficient mitochondria carrying either of two human mtDNA pathogenetic recessive mutations in the genes for tRNA^(Ile) and tRNA^(Leu(UUR)). Nearly all the hybrids exhibited normal mitochondrial protein synthesis and respiratory activity 10 to 14 days after fusion, but not earlier. Their results are in striking contrast with our previous observations, which indicated clearly that complementation between two different deleterious mtDNA mutations carried within distinct organelles in the same human cell could indeed occur, but was a rare phenomenon in the cells analyzed. We wish to point out that the discrepancy between the conclusions of the two laboratories does not concern the issue of whether mammalian mitochondria have the potential to fuse and mix their mtDNA and/or mtDNA products in vivo, an issue on which we do not disagree, but which rather has to do with the generality and frequency of this phenomenon and the importance of its control by the nucleus.
影响因子：
4
作者：
A. Santel;M. Fuller
通讯作者：
A. Santel;M. Fuller