Cell Biology Program, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, Box 390, New York, NY 10065, USA.
J Cell Sci. 2010 Nov 15;123(Pt 22):3849-55. doi: 10.1242/jcs.075119.
Mitochondria are required for numerous essential metabolic processes including the regulation of apoptosis; therefore, proper maintenance of the mitochondrial proteome is crucial. The protein-folding environment in mitochondria is challenged by organelle architecture, the presence of reactive oxygen species and the difficulties associated with assembly of the electron transport chain, which consists of components encoded by both the mitochondrial and the nuclear genomes. Mitochondria have dedicated molecular chaperones and proteases that promote proper protein folding, complex assembly and quality control. Work in cultured mammalian cells and Caenorhabditis elegans has yielded clues to the mechanisms linking perturbations in the protein-folding environment in the mitochondrial matrix to the expression of nuclear genes encoding mitochondrial proteins. Here, we review the current knowledge of this mitochondrial unfolded protein response (UPR(mt)), compare it with the better understood UPR of the endoplasmic reticulum and highlight its potential impact on development and disease.
线粒体对于许多基本的代谢过程是必需的，包括细胞凋亡的调节；因此，线粒体蛋白质组的适当维持至关重要。线粒体的蛋白质折叠环境受到细胞器结构、活性氧的存在以及电子传递链组装相关困难的挑战，而电子传递链由线粒体和核基因组共同编码的组件组成。线粒体有专门的分子伴侣和蛋白酶，可促进蛋白质的正确折叠、复合物的组装和质量控制。在培养的哺乳动物细胞和秀丽隐杆线虫中的研究工作为连接线粒体基质中蛋白质折叠环境的扰动与核基因编码线粒体蛋白的表达的机制提供了线索。在这里，我们综述了这一线粒体未折叠蛋白反应（UPR(mt)）的最新知识，将其与内质网中更为人所熟知的 UPR 进行了比较，并强调了其对发育和疾病的潜在影响。
