Analysis of Mitochondrial Morphology and Function

描述（由申请人提供）：线粒体是一种细胞器，负责ATP的生成、类固醇激素的合成、环境毒素的解毒，是细胞凋亡的主要目标。虽然线粒体膜的结构和线粒体的生化过程已经被很好地描述，但对细胞内线粒体的组织和分布在线粒体功能中的作用知之甚少。在细胞内，单个线粒体被组织成一个动态网状网络，统称为线粒体。虽然，控制线粒体形态的机制的基本原理已经在许多模型系统中得到了解决，最著名的是酵母，酿酒酵母。很少有研究表明这种组织对线粒体功能的影响。此外，在细胞类型多样性较大的高等后生动物中，线粒体组织的数据很少。许多肌病，包括肌萎缩性侧索硬化症、帕金森氏病、MELAS和Kearns-Sayre综合征，导致线粒体的改变或破坏。这些干扰是主要影响还是仅仅是次要影响尚不清楚。本提案的目的是通过使用果蝇模型系统来了解线粒体细胞内组织的控制机制。果蝇模型系统为本科生提供了出色的动手研究经验，允许许多不同的实验方法（遗传，生化和细胞）用于这种结构/功能研究。这个项目在研究实验室和教室之间架起了桥梁。线粒体氧化磷酸化和电子传递链的基本概念在每个生物学课程中都很早就教授过。本项目拟通过对线粒体形态的研究，向学生介绍结构与功能的关系：线粒体的结构与功能。

DESCRIPTION (provided by applicant): Mitochondria are the cellular organelles that are responsible for the generation of ATP, the synthesis of steroid hormones, the detoxification of environmental toxins, and are the primary target of apoptosis. Although the structure of the mitochondrial membrane and the biochemical processes of the mitochondria are well described, little is understood about the role that the organization and distribution of the mitochondria within the cell has in the function of the mitochondria. Within a cell, individual mitochondria are organized into a dynamic reticulate network collectively called the mitochondrion. Although, the rudiments of the mechanisms that govern mitochondrial morphology have been worked out in a number of model systems, most notably the yeast, Saccharomyces cerevisiae. Little work has been done to show the effects that this organization has in the mitochondrial function. Furthermore, little data are available in the organization of mitochondria in higher metazoans, which have greater diversity of cell type. Many myopathies including Amyotrophic Lateral Sclerosis, Parkinsons' Disease, MELAS, and Kearns-Sayre Syndrome, result in the alteration or disruption of the mitochondrion. Whether these disruptions are the primary effect or simply secondary effect is unclear. The goal of this proposal is to understand the mechanisms that govern the intracellular organization of the mitochondrion by using the Drosophila melanogaster model system. The Drosophila model system provides an outstanding hand-on research experience for undergraduate students allowing many different experimental approaches (genetic, biochemical and cellular) with which to use in this structure/function investigation. This project bridges the gap between the research laboratory and the classroom. The basic concepts of mitochondrial oxidative phosphorylation and electron transport chains are taught early in every biology curriculum. The project plans on using the study of mitochondria morphology to introduce to students the correlation between structure and function: mitochondrion structure and its function.