线粒体蛋白质量控制在氧化应激诱导的神经退行性疾病中的作用

Title:Role of Mitochondrial Protein Quality Control in Oxidative Stress-induced Neurodegenerative Diseases

Volume: 13 Issue: 2

Author(s): Giovanna Cenini and Wolfgang Voos

Affiliation:

关键词: 阿尔茨海默病，线粒体蛋白质的质量控制（mtPQC），氧化应激，帕金森病。

摘要: Proteins are constantly exposed to environmental stressors such as free radicals and heat shock leading to their misfolding and later to aggregation. In particular mitochondrial proteins are challenged by reactive oxygen species (ROS) due to the oxidative metabolism of the organelle. Protein aggregation has been associated with a wide variety of pathological conditions called proteopathies. However, for the maintenance of protein and cellular homeostasis, mitochondria have developed an elaborate protein quality control system consisting of chaperones and ATP-dependent proteases, specifically employed to rescue this organelle from damage due to the accumulation of misfolded proteins and toxic aggregates. Aging is characterized by a general decline of mitochondrial functions, correlating with a decrease in mitochondrial protein quality control activity and an increase of free radical production. In particular in age-related diseases like neurodegeneration, a correlation between mitochondrial damage and disease onset has been established. In this review we summarize the current knowledge about mitochondrial protein quality control mechanisms in mammalian cells, with a special emphasis on the role in oxidative stress and in neurodegenerative diseases.

Cite this article as:

Giovanna Cenini and Wolfgang Voos , Role of Mitochondrial Protein Quality Control in Oxidative Stress-induced Neurodegenerative Diseases, Current Alzheimer Research 2016; 13 (2) . https://dx.doi.org/10.2174/1567205012666150921103213