Tsang William Y, Lemire Bernard D
Canadian Institutes of Health Research Group in Membrane Protein Research, Department of Biochemistry, University of Alberta, 474 Medical Sciences Bldg., Edmonton, Alberta, Canada T6G 2H7.
Biochim Biophys Acta. 2003 Jul 14;1638(2):91-105. doi: 10.1016/s0925-4439(03)00079-6.
Mitochondria are essential organelles involved in energy metabolism via oxidative phosphorylation. They play a vital role in diverse biological processes such as aging and apoptosis. In humans, defects in the mitochondrial respiratory chain (MRC) are responsible for or associated with a bewildering variety of diseases. The nematode Caenorhabditis elegans is a simple animal and a powerful genetic and developmental model system. In this review, we discuss how the nematode model system has contributed to our understanding of mitochondrial dynamics, of the genetics and inheritance of the mitochondrial genome, and of the consequences of nuclear and mitochondrial DNA (mtDNA) mutations. Mitochondrial respiration is vital to energy metabolism but also to other aspects of multicellular life such as aging and development. We anticipate that further significant contributions to our understanding of mitochondrial function in animal biology are forthcoming with the C. elegans model system.
线粒体是通过氧化磷酸化参与能量代谢的重要细胞器。它们在衰老和细胞凋亡等多种生物学过程中发挥着至关重要的作用。在人类中,线粒体呼吸链(MRC)的缺陷导致或与各种各样令人困惑的疾病相关。线虫秀丽隐杆线虫是一种简单的动物,也是一个强大的遗传和发育模型系统。在这篇综述中,我们讨论了线虫模型系统如何有助于我们理解线粒体动力学、线粒体基因组的遗传学和遗传方式,以及核DNA和线粒体DNA(mtDNA)突变的后果。线粒体呼吸对于能量代谢至关重要,但对于多细胞生命的其他方面,如衰老和发育也很重要。我们预计,秀丽隐杆线虫模型系统将对我们理解动物生物学中线粒体功能做出进一步的重大贡献。