Richard C. Scarpulla is at the Department of Cell and Molecular Biology, Northwestern Medical School, Chicago, IL 60611, USA.
Trends Cardiovasc Med. 1996 Feb;6(2):39-45. doi: 10.1016/1050-1738(95)00129-8.
Mitochondrial oxidative pathways can be adversely affected by mutations in both nuclear and mitochondrial genomes. Recent evidence indicates that cardiac impairment is an important clinical feature of mitochondrial diseases resulting from such mutations. Understanding the regulatory interplay between nuclear and mitochondrial genetic systems may yield new insights into human genetic defects affecting cardiac function. Nuclear respiratory factors (NRF-1 and NRF-2) are transcriptional activators that act on a significant subset of nuclear genes required for mitochondrial respiration. These factors most likely participate in nuclear-mitochondrial interactions by helping to coordinate the synthesis of respiratory chain subunits with components of the mitochondrial transcription, replication, and heme biosynthetic machinery. Thus, NRFs and related factors are likely contributors to the nuclear control of mitochondrial energy production that is essential for normal myocardial function.
线粒体氧化途径可能会受到核基因组和线粒体基因组突变的不利影响。最近的证据表明，心脏损伤是由这些突变引起的线粒体疾病的一个重要临床特征。了解核和线粒体遗传系统之间的调节相互作用，可能会为影响心脏功能的人类遗传缺陷提供新的见解。核呼吸因子（NRF-1 和 NRF-2）是转录激活因子，它们作用于线粒体呼吸所需的一组重要核基因上。这些因子很可能通过帮助协调呼吸链亚基与线粒体转录、复制和血红素生物合成机制的成分的合成，参与核-线粒体相互作用。因此，NRF 及其相关因子可能是核控制线粒体能量产生的重要贡献者，线粒体能量产生对于正常心肌功能是必不可少的。
