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Role of PGC-1α in Mitochondrial Quality Control in Neurodegenerative Diseases

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Abstract

As one of the major cell organelles responsible for ATP production, it is important that neurons maintain mitochondria with structural and functional integrity; this is especially true for neurons with high metabolic requirements. When mitochondrial damage occurs, mitochondria are able to maintain a steady state of functioning through molecular and organellar quality control, thus ensuring neuronal function. And when mitochondrial quality control (MQC) fails, mitochondria mediate apoptosis. An apparently key molecule in MQC is the transcriptional coactivator peroxisome proliferator activated receptor γ coactivator-1α (PGC-1α). Recent findings have demonstrated that upregulation of PGC-1α expression in neurons can modulate MQC to prevent mitochondrial dysfunction in certain in vivo and in vitro aging or neurodegenerative encephalopathy models, such as Huntington’s disease, Alzheimer’s disease, and Parkinson’s disease. Because mitochondrial function and quality control disorders are the basis of pathogenesis in almost all neurodegenerative diseases (NDDs), the role of PGC-1α may make it a viable entry point for the treatment of such diseases. This review focuses on multi-level MQC in neurons, as well as the regulation of MQC by PGC-1α in these major NDDs.

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Funding

Funding was supported by Jilin Provincial Research Foundation for the Development of Science and Technology Projects (Grant no. 20190201152JC).

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  1. Qi Zhang and Yu-Hong Lei contributed equally to this work.

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  1. Department of Intensive Care Unit, The First Hospital of Jilin University, Changchun, 130021, China

    Qi Zhang

  2. Department of Neurosurgery, The First Hospital of Jilin University, Changchun, 130021, China

    Yu-Hong Lei, Jue-Pu Zhou, Ye-Ye Hou, Zheng Wan, Hong-Lei Wang & Hao Meng

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Zhang, Q., Lei, YH., Zhou, JP. et al. Role of PGC-1α in Mitochondrial Quality Control in Neurodegenerative Diseases. Neurochem Res 44, 2031–2043 (2019). https://doi.org/10.1007/s11064-019-02858-6

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