首页 | 本学科首页   官方微博 | 高级检索  
   检索      


Complexity and tissue specificity of the mitochondrial respiratory chain
Authors:Roderick A Capaldi  Diego Gonzalez Halphen  Yu-Zhong Zhang  Wayne Yanamura
Institution:(1) Institute of Molecular Biology, University of Oregon, 97403 Eugene, Oregon
Abstract:There is a renewed interest in the structure and functioning of the mitochondrial respiratory chain with the realization that a number of genetic disorders result from defects in mitochondrial electron transfer. These so-called mitochondrial myopathies include diseases of muscle, heart, and brain. The respiratory chain can be fractionated into four large multipeptide complexes, an NADH ubiquinone reductase (complex I), succinate ubiquinone reductase (complex II), ubiquinol oxidoreductase (complex III), and cytochromec oxidase (complex IV). Mitochondrial myopathies involving each of these complexes have been described. This review summarizes compositional and structural data on the respiratory chain proteins and describes the arrangement of these complexes in the mitochondrial inner membrane. This biochemical information is provided as a framework for the diagnosis and molecular characterization of mitochondrial diseases.
Keywords:Mitochondrial myopathies  electron transfer chain  tissue specificity  electron microscopy  protein sequences
本文献已被 SpringerLink 等数据库收录!
设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号