The mitochondrial UPR - protecting organelle protein homeostasis
The mitochondrial UPR - protecting organelle protein homeostasis
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DOI：
10.1242/jcs.075119
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发表时间：
2010-11-15
影响因子：
4
通讯作者：
Ron, David
中科院分区：
文献类型：
--
作者：
Haynes, Cole M.;Ron, David
线粒体是许多必需的代谢过程所必需的，包括细胞凋亡的调节;因此，线粒体蛋白质组的适当维护至关重要。线粒体中的蛋白质折叠环境受到细胞器结构、活性氧物质的存在以及与电子传递链组装相关的困难的挑战，电子传递链由线粒体和核基因组编码的组分组成。线粒体具有专用的分子伴侣和蛋白酶，其促进适当的蛋白质折叠、复合物组装和质量控制。在培养的哺乳动物细胞和秀丽隐杆线虫中的工作已经产生了将线粒体基质中的蛋白质折叠环境中的扰动与编码线粒体蛋白的核基因的表达联系起来的机制的线索。在这里，我们回顾了目前的知识，这种线粒体未折叠蛋白反应（UPRmt），比较它与更好地理解UPR的内质网，并强调其对发展和疾病的潜在影响。
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 (UPRmt), compare it with the better understood UPR of the endoplasmic reticulum and highlight its potential impact on development and disease.