Abstract
Neurodegenerative diseases are disorders that are characterized by a progressive decline of motor and/or cognitive functions caused by the selective degeneration and loss of neurons within the central nervous system. The most common neurodegenerative diseases are Alzheimer’s disease (AD), Parkinson’s disease (PD), and Huntington’s disease (HD). Neurons have high energy demands, and dysregulation of mitochondrial quality and function is an important cause of neuronal degeneration. Mitochondrial quality control plays an important role in maintaining mitochondrial integrity and ensuring normal mitochondrial function; thus, defects in mitochondrial quality control are also significant causes of neurodegenerative diseases. The mitochondrial deacetylase SIRT3 has been found to have a large effect on mitochondrial function. Recent studies have also shown that SIRT3 has a role in mitochondrial quality control, including in the refolding or degradation of misfolded/unfolded proteins, mitochondrial dynamics, mitophagy, and mitochondrial biogenesis, all of which are affected in neurodegenerative diseases.
Highlights
Neurodegenerative diseases involve mitochondrial dysfunction caused by various factors, which leads to progressive degeneration, apoptosis, or necrosis of neurons (Srivastava and Yadav, 2016)
Previous studies have found that a dysregulation of mitochondrial function is an important cause of neuronal degeneration, and recent studies have shown that mitochondrial quality control has a role in maintaining mitochondrial integrity and ensuring normal mitochondrial function (Palikaras and Tavernarakis, 2014; Ni et al, 2015; Palikaras et al, 2015)
Mitochondrial function is regulated by a variety of enzymes, and in this review, we have focused on the important mitochondrial NAD-dependent deacetylase sirtuin-3 (SIRT3; Finley et al, 2011)
Summary
Neurodegenerative diseases involve mitochondrial dysfunction caused by various factors, which leads to progressive degeneration, apoptosis, or necrosis of neurons (Srivastava and Yadav, 2016). Previous studies have found that a dysregulation of mitochondrial function is an important cause of neuronal degeneration, and recent studies have shown that mitochondrial quality control has a role in maintaining mitochondrial integrity and ensuring normal mitochondrial function (Palikaras and Tavernarakis, 2014; Ni et al, 2015; Palikaras et al, 2015). This review summarizes recent research into mitochondrial quality control and the role of SIRT3 in mitochondrial function, and further illustrates the effects of SIRT3 on mitochondrial quality control in the neurodegenerative diseases AD, PD, and HD. This will provide a reference for exploring the relationship between mitochondrial function and quality, as well as for seeking new targets for the treatment of neurodegenerative diseases
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