Zee Jennifer M, Glerum D Moira
Department of Medical Genetics, University of Alberta, Edmonton, AB T6G 0M2, Canada.
Biochem Cell Biol. 2006 Dec;84(6):859-69. doi: 10.1139/o06-201.
The biogenesis of the inner mitochondrial membrane enzyme cytochrome c oxidase (COX) is a complex process that requires the actions of ancillary proteins, collectively called assembly factors. Studies with the yeast Saccharomyces cerevisiae have provided considerable insight into the COX assembly pathway and have proven to be a fruitful model for understanding the molecular bases for inherited COX deficiencies in humans. In this review, we focus on critical steps in the COX assembly pathway. These processes are conserved from yeast to humans and are known to be involved in the etiology of human COX deficiencies. The contributions from our studies in yeast suggest that this organism remains an excellent model system for delineating the molecular mechanisms underlying COX assembly defects in humans. Current progress suggests that a complete picture of COX assembly will be achieved in the near future.
线粒体内膜酶细胞色素c氧化酶（COX）的生物合成是一个复杂的过程，需要辅助蛋白的作用，这些辅助蛋白统称为装配因子。对酿酒酵母的研究为COX装配途径提供了相当多的见解，并且已被证明是理解人类遗传性COX缺陷分子基础的一个富有成果的模型。在这篇综述中，我们关注COX装配途径中的关键步骤。这些过程从酵母到人类都是保守的，并且已知与人类COX缺陷的病因有关。我们在酵母中的研究表明，这种生物体仍然是描绘人类COX装配缺陷潜在分子机制的优秀模型系统。目前的进展表明，在不久的将来将实现对COX装配的完整认识。
