表征电子传递链：结构方法。

Characterizing the Electron Transport Chain: Structural Approach.

机构信息

Key Laboratory of Laboratory Medicine, Ministry of Education, Zhejiang Provincial Key Laboratory of Medical Genetics, College of Laboratory Medicine and Life Sciences, Wenzhou Medical University, Wenzhou, Zhejiang, China.

Department of Cell Systems and Anatomy, University of Texas Health San Antonio, San Antonio, TX, USA.

出版信息

Methods Mol Biol. 2022;2497:107-115. doi: 10.1007/978-1-0716-2309-1_7.

DOI:10.1007/978-1-0716-2309-1_7

PMID:35771438

Abstract

The mitochondrial respiratory chain which carries out the oxidative phosphorylation (OXPHOS) consists of five multi-subunit protein complexes. Emerging evidences suggest that the supercomplexes which further consist of multiple respiratory complexes play important role in regulating OXPHOS function. Dysfunction of the respiratory chain and its regulation has been implicated in various human diseases including neurodegenerative diseases and muscular disorders. Many mouse models have been established which exhibit mitochondrial defects in brain and muscles. Protocols presented here aim to help to analyze the structures of mitochondrial respiratory chain which include the preparation of the tissue samples, isolation of mitochondrial membrane proteins, and analysis of their respiratory complexes by Blue Native Polyacrylamide Gel Electrophoresis (BN-PAGE) in particular.

摘要

线粒体呼吸链执行氧化磷酸化（OXPHOS），由五个多亚基蛋白复合物组成。新兴证据表明，由多个呼吸复合物进一步组成的超复合物在调节 OXPHOS 功能方面发挥着重要作用。呼吸链的功能障碍及其调节与包括神经退行性疾病和肌肉疾病在内的各种人类疾病有关。已经建立了许多表现出脑和肌肉中线粒体缺陷的小鼠模型。本文提供的方案旨在帮助分析线粒体呼吸链的结构，包括组织样品的制备、线粒体膜蛋白的分离，以及通过 Blue Native Polyacrylamide Gel Electrophoresis（BN-PAGE）特别分析它们的呼吸复合物。