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Biomolecules
Special Issues
Mitochondria and Central Nervous System Disorders II
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Mitochondria and Central Nervous System Disorders II

A special issue of Biomolecules (ISSN 2218-273X). This special issue belongs to the section "Molecular Medicine".

Deadline for manuscript submissions: closed (15 September 2023) | Viewed by 27821

Special Issue Editors

Dr. Camilla Bean

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Guest Editor
Department of Medicine, University of Udine, 33100 Udine, Italy
Interests: mitochondria; metabolism; transcriptomics; epigenetics
Dr. Marta Zaninello

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Guest Editor
1. Institute of Human Genetics, University of Cologne, Cologne, Germany
2. Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases, University of Cologne, Cologne, Germany
Interests: mitochondria; translation; autophagy; neurons

Special Issue Information

Dear Colleagues,

Following a very successful first run, we are pleased to announce the launch of a second edition of our Special Issue on “Mitochondria and Central Nervous System Disorders II”.

In the past several decades, a mitochondria-centric vision has developed in the fields of cell, organ, and organismal physiology, demonstrating exponential growth. This is likely due to the number of findings highlighting the contribution of these organelles to cell/tissue bioenergetics, death programmes, and metabolism. Dysfunctional mitochondria or dysfunctional mitochondria dynamics (a term that includes processes dictating the morphology of these organelles, their subcellular distribution/transport, or their interaction with other organelles, consequently influencing their function) have been linked to many pathological conditions, widespread among the entire human body. However, these alterations appear to more strongly affect the highly specialized and delicate cells of the central nervous system (CNS), contributing to the onset of a variety of diseases, ranging from rare childhood disorders (e.g., Leigh syndrome or mitochondrial encephalopathy with lactic acidosis and stroke-like episodes) to more common age-related neurodegenerative conditions (e.g., dementia, Alzheimer’s disease, and Parkinson’s disease).

This Special Issue is designed to emphasize the link between the (dys)function of mitochondria and CNS disorders, likely highlighting common or discrepant mechanisms underlying them. In this regard, we would like to invite review articles which address the above-mentioned topics or original research papers providing new evidence on the mitochondria–CNS pathological connection.

We look forward to reading your contributions.

Dr. Camilla Bean
Dr. Marta Zaninello
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomolecules is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • mitochondria
  • dementia
  • neurodegeneration
  • neurodevelopment
  • central nervous system

Related Special Issue

Published Papers (7 papers)

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Review

29 pages, 2676 KiB  
Review
Cyclophilin D in Mitochondrial Dysfunction: A Key Player in Neurodegeneration?
by Gabriele Coluccino, Valentina Pia Muraca, Alessandra Corazza and Giovanna Lippe
Biomolecules 2023, 13(8), 1265; https://doi.org/10.3390/biom13081265 - 18 Aug 2023
Viewed by 1498
Abstract
Mitochondrial dysfunction plays a pivotal role in numerous complex diseases. Understanding the molecular mechanisms by which the “powerhouse of the cell” turns into the “factory of death” is an exciting yet challenging task that can unveil new therapeutic targets. The mitochondrial matrix protein [...] Read more.
Mitochondrial dysfunction plays a pivotal role in numerous complex diseases. Understanding the molecular mechanisms by which the “powerhouse of the cell” turns into the “factory of death” is an exciting yet challenging task that can unveil new therapeutic targets. The mitochondrial matrix protein CyPD is a peptidylprolyl cis-trans isomerase involved in the regulation of the permeability transition pore (mPTP). The mPTP is a multi-conductance channel in the inner mitochondrial membrane whose dysregulated opening can ultimately lead to cell death and whose involvement in pathology has been extensively documented over the past few decades. Moreover, several mPTP-independent CyPD interactions have been identified, indicating that CyPD could be involved in the fine regulation of several biochemical pathways. To further enrich the picture, CyPD undergoes several post-translational modifications that regulate both its activity and interaction with its clients. Here, we will dissect what is currently known about CyPD and critically review the most recent literature about its involvement in neurodegenerative disorders, focusing on Alzheimer’s Disease and Parkinson’s Disease, supporting the notion that CyPD could serve as a promising therapeutic target for the treatment of such conditions. Notably, significant efforts have been made to develop CyPD-specific inhibitors, which hold promise for the treatment of such complex disorders. Full article
(This article belongs to the Special Issue Mitochondria and Central Nervous System Disorders II)
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16 pages, 364 KiB  
Review
Connecting Dots between Mitochondrial Dysfunction and Depression
by Mehtab Khan, Yann Baussan and Etienne Hebert-Chatelain
Biomolecules 2023, 13(4), 695; https://doi.org/10.3390/biom13040695 - 20 Apr 2023
Cited by 9 | Viewed by 3900
Abstract
Mitochondria are the prime source of cellular energy, and are also responsible for important processes such as oxidative stress, apoptosis and Ca2+ homeostasis. Depression is a psychiatric disease characterized by alteration in the metabolism, neurotransmission and neuroplasticity. In this manuscript, we summarize [...] Read more.
Mitochondria are the prime source of cellular energy, and are also responsible for important processes such as oxidative stress, apoptosis and Ca2+ homeostasis. Depression is a psychiatric disease characterized by alteration in the metabolism, neurotransmission and neuroplasticity. In this manuscript, we summarize the recent evidence linking mitochondrial dysfunction to the pathophysiology of depression. Impaired expression of mitochondria-related genes, damage to mitochondrial membrane proteins and lipids, disruption of the electron transport chain, higher oxidative stress, neuroinflammation and apoptosis are all observed in preclinical models of depression and most of these parameters can be altered in the brain of patients with depression. A deeper knowledge of the depression pathophysiology and the identification of phenotypes and biomarkers with respect to mitochondrial dysfunction are needed to help early diagnosis and the development of new treatment strategies for this devastating disorder. Full article
(This article belongs to the Special Issue Mitochondria and Central Nervous System Disorders II)
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Graphical abstract

21 pages, 968 KiB  
Review
Caenorhabditis elegans as a Model System to Study Human Neurodegenerative Disorders
by Antonis Roussos, Katerina Kitopoulou, Fivos Borbolis and Konstantinos Palikaras
Biomolecules 2023, 13(3), 478; https://doi.org/10.3390/biom13030478 - 05 Mar 2023
Cited by 6 | Viewed by 4926
Abstract
In recent years, advances in science and technology have improved our quality of life, enabling us to tackle diseases and increase human life expectancy. However, longevity is accompanied by an accretion in the frequency of age-related neurodegenerative diseases, creating a growing burden, with [...] Read more.
In recent years, advances in science and technology have improved our quality of life, enabling us to tackle diseases and increase human life expectancy. However, longevity is accompanied by an accretion in the frequency of age-related neurodegenerative diseases, creating a growing burden, with pervasive social impact for human societies. The cost of managing such chronic disorders and the lack of effective treatments highlight the need to decipher their molecular and genetic underpinnings, in order to discover new therapeutic targets. In this effort, the nematode Caenorhabditis elegans serves as a powerful tool to recapitulate several disease-related phenotypes and provides a highly malleable genetic model that allows the implementation of multidisciplinary approaches, in addition to large-scale genetic and pharmacological screens. Its anatomical transparency allows the use of co-expressed fluorescent proteins to track the progress of neurodegeneration. Moreover, the functional conservation of neuronal processes, along with the high homology between nematode and human genomes, render C. elegans extremely suitable for the study of human neurodegenerative disorders. This review describes nematode models used to study neurodegeneration and underscores their contribution in the effort to dissect the molecular basis of human diseases and identify novel gene targets with therapeutic potential. Full article
(This article belongs to the Special Issue Mitochondria and Central Nervous System Disorders II)
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22 pages, 1726 KiB  
Review
Mitochondrial Neurodegeneration: Lessons from Drosophila melanogaster Models
by Michele Brischigliaro, Erika Fernandez-Vizarra and Carlo Viscomi
Biomolecules 2023, 13(2), 378; https://doi.org/10.3390/biom13020378 - 16 Feb 2023
Cited by 5 | Viewed by 4816
Abstract
The fruit fly—i.e., Drosophila melanogaster—has proven to be a very useful model for the understanding of basic physiological processes, such as development or ageing. The availability of straightforward genetic tools that can be used to produce engineered individuals makes this model extremely [...] Read more.
The fruit fly—i.e., Drosophila melanogaster—has proven to be a very useful model for the understanding of basic physiological processes, such as development or ageing. The availability of straightforward genetic tools that can be used to produce engineered individuals makes this model extremely interesting for the understanding of the mechanisms underlying genetic diseases in physiological models. Mitochondrial diseases are a group of yet-incurable genetic disorders characterized by the malfunction of the oxidative phosphorylation system (OXPHOS), which is the highly conserved energy transformation system present in mitochondria. The generation of D. melanogaster models of mitochondrial disease started relatively recently but has already provided relevant information about the molecular mechanisms and pathological consequences of mitochondrial dysfunction. Here, we provide an overview of such models and highlight the relevance of D. melanogaster as a model to study mitochondrial disorders. Full article
(This article belongs to the Special Issue Mitochondria and Central Nervous System Disorders II)
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18 pages, 2230 KiB  
Review
Exogenous Players in Mitochondria-Related CNS Disorders: Viral Pathogens and Unbalanced Microbiota in the Gut-Brain Axis
by Irene Righetto, Matteo Gasparotto, Laura Casalino, Marcella Vacca and Francesco Filippini
Biomolecules 2023, 13(1), 169; https://doi.org/10.3390/biom13010169 - 13 Jan 2023
Cited by 4 | Viewed by 3611
Abstract
Billions of years of co-evolution has made mitochondria central to the eukaryotic cell and organism life playing the role of cellular power plants, as indeed they are involved in most, if not all, important regulatory pathways. Neurological disorders depending on impaired mitochondrial function [...] Read more.
Billions of years of co-evolution has made mitochondria central to the eukaryotic cell and organism life playing the role of cellular power plants, as indeed they are involved in most, if not all, important regulatory pathways. Neurological disorders depending on impaired mitochondrial function or homeostasis can be caused by the misregulation of “endogenous players”, such as nuclear or cytoplasmic regulators, which have been treated elsewhere. In this review, we focus on how exogenous agents, i.e., viral pathogens, or unbalanced microbiota in the gut-brain axis can also endanger mitochondrial dynamics in the central nervous system (CNS). Neurotropic viruses such as Herpes, Rabies, West-Nile, and Polioviruses seem to hijack neuronal transport networks, commandeering the proteins that mitochondria typically use to move along neurites. However, several neurological complications are also associated to infections by pandemic viruses, such as Influenza A virus and SARS-CoV-2 coronavirus, representing a relevant risk associated to seasonal flu, coronavirus disease-19 (COVID-19) and “Long-COVID”. Emerging evidence is depicting the gut microbiota as a source of signals, transmitted via sensory neurons innervating the gut, able to influence brain structure and function, including cognitive functions. Therefore, the direct connection between intestinal microbiota and mitochondrial functions might concur with the onset, progression, and severity of CNS diseases. Full article
(This article belongs to the Special Issue Mitochondria and Central Nervous System Disorders II)
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43 pages, 4486 KiB  
Review
Linking the Amyloid, Tau, and Mitochondrial Hypotheses of Alzheimer’s Disease and Identifying Promising Drug Targets
by Zdeněk Fišar
Biomolecules 2022, 12(11), 1676; https://doi.org/10.3390/biom12111676 - 11 Nov 2022
Cited by 23 | Viewed by 4808
Abstract
Damage or loss of brain cells and impaired neurochemistry, neurogenesis, and synaptic and nonsynaptic plasticity of the brain lead to dementia in neurodegenerative diseases, such as Alzheimer’s disease (AD). Injury to synapses and neurons and accumulation of extracellular amyloid plaques and intracellular neurofibrillary [...] Read more.
Damage or loss of brain cells and impaired neurochemistry, neurogenesis, and synaptic and nonsynaptic plasticity of the brain lead to dementia in neurodegenerative diseases, such as Alzheimer’s disease (AD). Injury to synapses and neurons and accumulation of extracellular amyloid plaques and intracellular neurofibrillary tangles are considered the main morphological and neuropathological features of AD. Age, genetic and epigenetic factors, environmental stressors, and lifestyle contribute to the risk of AD onset and progression. These risk factors are associated with structural and functional changes in the brain, leading to cognitive decline. Biomarkers of AD reflect or cause specific changes in brain function, especially changes in pathways associated with neurotransmission, neuroinflammation, bioenergetics, apoptosis, and oxidative and nitrosative stress. Even in the initial stages, AD is associated with Aβ neurotoxicity, mitochondrial dysfunction, and tau neurotoxicity. The integrative amyloid-tau-mitochondrial hypothesis assumes that the primary cause of AD is the neurotoxicity of Aβ oligomers and tau oligomers, mitochondrial dysfunction, and their mutual synergy. For the development of new efficient AD drugs, targeting the elimination of neurotoxicity, mutual potentiation of effects, and unwanted protein interactions of risk factors and biomarkers (mainly Aβ oligomers, tau oligomers, and mitochondrial dysfunction) in the early stage of the disease seems promising. Full article
(This article belongs to the Special Issue Mitochondria and Central Nervous System Disorders II)
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Graphical abstract

16 pages, 1000 KiB  
Review
Metabolic Regulation of Mitochondrial Protein Biogenesis from a Neuronal Perspective
by Jara Tabitha Hees and Angelika Bettina Harbauer
Biomolecules 2022, 12(11), 1595; https://doi.org/10.3390/biom12111595 - 29 Oct 2022
Cited by 11 | Viewed by 2624
Abstract
Neurons critically depend on mitochondria for ATP production and Ca2+ buffering. They are highly compartmentalized cells and therefore a finely tuned mitochondrial network constantly adapting to the local requirements is necessary. For neuronal maintenance, old or damaged mitochondria need to be degraded, [...] Read more.
Neurons critically depend on mitochondria for ATP production and Ca2+ buffering. They are highly compartmentalized cells and therefore a finely tuned mitochondrial network constantly adapting to the local requirements is necessary. For neuronal maintenance, old or damaged mitochondria need to be degraded, while the functional mitochondrial pool needs to be replenished with freshly synthesized components. Mitochondrial biogenesis is known to be primarily regulated via the PGC-1α-NRF1/2-TFAM pathway at the transcriptional level. However, while transcriptional regulation of mitochondrial genes can change the global mitochondrial content in neurons, it does not explain how a morphologically complex cell such as a neuron adapts to local differences in mitochondrial demand. In this review, we discuss regulatory mechanisms controlling mitochondrial biogenesis thereby making a case for differential regulation at the transcriptional and translational level. In neurons, additional regulation can occur due to the axonal localization of mRNAs encoding mitochondrial proteins. Hitchhiking of mRNAs on organelles including mitochondria as well as contact site formation between mitochondria and endolysosomes are required for local mitochondrial biogenesis in axons linking defects in any of these organelles to the mitochondrial dysfunction seen in various neurological disorders. Full article
(This article belongs to the Special Issue Mitochondria and Central Nervous System Disorders II)
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