Mitochondrial dysfunction in the development and progression of neurodegenerative diseases

Johnson, Joseph Taber; Mercado-Ayón, Elizabeth; Mercado-Ayon, Yesica; Dong, Yi; Halawani, Sarah; Ngaba, Lucie; Lynch, David R.

doi:10.1016/j.abb.2020.108698

Cited by 158 publications

(103 citation statements)

References 139 publications

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“…The mitochondrial defect that causes energy deficiency in neurons is a major pathological feature of most neurodegenerative diseases and has been extensively reviewed recently (Monzio Compagnoni et al, 2020 ; Yan et al, 2020 ; Goyal and Chaturvedi, 2021 ; Johnson et al, 2021 ; Lee et al, 2021 ; Madruga et al, 2021 ; Nakagawa and Yamada, 2021 ). We have previously reviewed the evidence linking metabolic impairment and mitochondrial defect in neurodegenerative diseases (Liu and Chern, 2015 ; Liu et al, 2017 ).…”

Section: Impaired Translational Control Resulting From Energy Dysfunction In Neurodegenerative Diseasesmentioning

confidence: 99%

Contribution of Energy Dysfunction to Impaired Protein Translation in Neurodegenerative Diseases

Liu

Chern

2021

Front. Cell. Neurosci.

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Impaired energy homeostasis and aberrant translational control have independently been implicated in the pathogenesis of neurodegenerative diseases. AMP kinase (AMPK), regulated by the ratio of cellular AMP and ATP, is a major gatekeeper for cellular energy homeostasis. Abnormal regulation of AMPK has been reported in several neurodegenerative diseases, including Alzheimer’s disease (AD) and amyotrophic lateral sclerosis (ALS). Most importantly, AMPK activation is known to suppress the translational machinery by inhibiting the mechanistic target of rapamycin complex 1 (mTORC1), activating translational regulators, and phosphorylating nuclear transporter factors. In this review, we describe recent findings on the emerging role of protein translation impairment caused by energy dysregulation in neurodegenerative diseases.

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Section: Impaired Translational Control Resulting From Energy Dysfunction In Neurodegenerative Diseasesmentioning

confidence: 99%

Contribution of Energy Dysfunction to Impaired Protein Translation in Neurodegenerative Diseases

Liu

Chern

2021

Front. Cell. Neurosci.

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“…Mitochondria are the main energy suppliers of the cell. The maintenance of functional, active mitochondria ensures adequate energy production, which is essential for neuron survival and the prevention of degenerative processes that lead to neurodegenerative diseases and aging [ 24 ]. Thus, factors that ensure and reinforce energy production are crucial regulators of neuronal viability and function.…”

Section: Discussionmentioning

confidence: 99%

“…It is also widely accepted that neurons critically depend on mitochondrial function to ensure membrane excitability and to execute the complex processes of neurotransmission and plasticity [ 23 ]. Thus, mitochondrial dysfunction which is accompanied with increased ROS production and oxidative damage is associated with numerous neurodegenerative disorders [ 24 ].…”

Section: Introductionmentioning

confidence: 99%

Anti-Apoptotic and Antioxidant Activities of the Mitochondrial Estrogen Receptor Beta in N2A Neuroblastoma Cells

Tsialtas

Georgantopoulos

Karipidou

et al. 2021

IJMS

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Estrogens are steroid hormones that play a crucial role in the regulation of the reproductive and non-reproductive system physiology. Among non-reproductive systems, the nervous system is mainly affected by estrogens due to their antioxidant, anti-apoptotic, and anti-inflammatory activities, which are mediated by membranous and nuclear estrogen receptors, and also by non-estrogen receptor-associated estrogen actions. Neuronal viability and functionality are also associated with the maintenance of mitochondrial functions. Recently, the localization of estrogen receptors, especially estrogen receptor beta, in the mitochondria of many types of neuronal cells is documented, indicating the direct involvement of the mitochondrial estrogen receptor beta (mtERβ) in the maintenance of neuronal physiology. In this study, cell lines of N2A cells stably overexpressing a mitochondrial-targeted estrogen receptor beta were generated and further analyzed to study the direct involvement of mtERβ in estrogen neuroprotective antioxidant and anti-apoptotic actions. Results from this study revealed that the presence of estrogen receptor beta in mitochondria render N2A cells more resistant to staurosporine- and H2O2-induced apoptotic stimuli, as indicated by the reduced activation of caspase-9 and -3, the increased cell viability, the increased ATP production, and the increased resistance to mitochondrial impairment in the presence or absence of 17-β estradiol (E2). Thus, the direct involvement of mtERβ in antioxidant and anti-apoptotic activities is documented, rendering mtERβ a promising therapeutic target for mitochondrial dysfunction-associated degenerative diseases.

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“…Mitochondrial abnormality causes a variety of neurological diseases, such as ischemic brain disease, Parkinson’s disease, Alzheimer’s disease, stroke and mitochondrial encephalopathy [ 16 , 17 , 18 , 19 , 20 ]. However, their etiology and the role of mitochondria in the pathogenesis have not yet been clearly elucidated.…”

Section: Mitochondrial Dysfunction and Diseasementioning

confidence: 99%

Mitochondrial Transplantation as a Novel Therapeutic Strategy for Mitochondrial Diseases

Park

Lee

et al. 2021

IJMS

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Mitochondria are the major source of intercellular bioenergy in the form of ATP. They are necessary for cell survival and play many essential roles such as maintaining calcium homeostasis, body temperature, regulation of metabolism and apoptosis. Mitochondrial dysfunction has been observed in variety of diseases such as cardiovascular disease, aging, type 2 diabetes, cancer and degenerative brain disease. In other words, the interpretation and regulation of mitochondrial signals has the potential to be applied as a treatment for various diseases caused by mitochondrial disorders. In recent years, mitochondrial transplantation has increasingly been a topic of interest as an innovative strategy for the treatment of mitochondrial diseases by augmentation and replacement of mitochondria. In this review, we focus on diseases that are associated with mitochondrial dysfunction and highlight studies related to the rescue of tissue-specific mitochondrial disorders. We firmly believe that mitochondrial transplantation is an optimistic therapeutic approach in finding a potentially valuable treatment for a variety of mitochondrial diseases.

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Mitochondrial dysfunction in the development and progression of neurodegenerative diseases

Cited by 158 publications

References 139 publications

Contribution of Energy Dysfunction to Impaired Protein Translation in Neurodegenerative Diseases

Contribution of Energy Dysfunction to Impaired Protein Translation in Neurodegenerative Diseases

Anti-Apoptotic and Antioxidant Activities of the Mitochondrial Estrogen Receptor Beta in N2A Neuroblastoma Cells

Mitochondrial Transplantation as a Novel Therapeutic Strategy for Mitochondrial Diseases

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