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Dynamics of Human Mitochondrial Complex I Assembly: Implications for Neurodegenerative Diseases.


ABSTRACT: Neurons are extremely energy demanding cells and highly dependent on the mitochondrial oxidative phosphorylation (OXPHOS) system. Mitochondria generate the energetic potential via the respiratory complexes I to IV, which constitute the electron transport chain (ETC), together with complex V. These redox reactions release energy in the form of ATP and also generate reactive oxygen species (ROS) that are involved in cell signaling but can eventually lead to oxidative stress. Complex I (CI or NADH:ubiquinone oxidoreductase) is the largest ETC enzyme, containing 44 subunits and the main contributor to ROS production. In recent years, the structure of the CI has become available and has provided new insights into CI assembly. A number of chaperones have been identified in the assembly and stabi

SUBMITTER: Giachin G 

PROVIDER: S-EPMC4992684 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature