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Cells
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Mitochondrial Dysfunction in Neurological Disorders: Molecular Mechanisms and Potential Points...
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Mitochondrial Dysfunction in Neurological Disorders: Molecular Mechanisms and Potential Points for Intervention

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cells of the Nervous System".

Deadline for manuscript submissions: closed (15 August 2023) | Viewed by 11797

Special Issue Editors

Dr. Anna Wilkaniec

E-Mail Website
Guest Editor
Mossakowski Medical Research Institute, Polish Academy of Sciences, Warsaw, Poland
Interests: purinergic signaling, α-Synuclein; mitochondria dysfunction; neuroinflammation; neurodegeneration
Dr. Agata Adamczyk

E-Mail Website
Guest Editor
Mossakowski Medical Research Institute, Polish Academy of Sciences, Warsaw, Poland
Interests: autism spectrum disorders; metabolic disturbances; mitochondrial dysfunction; neurodegeneration
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Mitochondria play a major role in the regulation of cellular bioenergetics as well as various non-energetic processes, like cell death mechanisms, generation of free radicals and antioxidative defense, immune response, and regulation of calcium homeostasis. Therefore, abnormal mitochondria signaling may be an important link between various disease-associated factors common to, and critical in, different central nervous system (CNS) diseases: from neurodevelopmental disorders, that occur early in life and persist until adulthood, to age-related neurodegenerative diseases. Understanding the exact position of mitochondrial dysfunction in the deleterious feed-forward loop critical for the development and progression of CNS diseases may help design successful therapeutic strategies for several fatal syndromes.

In this Special Issue, we cordially invite you to contribute, either in the form of original research articles, or reviews on all aspects related to the topic, describing mechanistic, functional, cellular, biochemical, or general aspects of mitochondrial dysfunction in neurological disorders. Relevant topics include, but are not limited to:

  • Ageing
  • Maternal immune activation
  • Epigenetic factors
  • Misfolded protein spread
  • Glial pathology
  • Inflammation
  • In vitro and in vivo models
  • Microbiota
  • Diagnostics
  • Bioimaging
  • Translational medicine

Dr. Anna Wilkaniec
Dr. Agata Adamczyk
Guest Editors

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Published Papers (3 papers)

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Research

29 pages, 7655 KiB  
Article
The Beneficial Effect of Mitochondrial Transfer Therapy in 5XFAD Mice via Liver–Serum–Brain Response
by Sahar Sweetat, Keren Nitzan, Nir Suissa, Yael Haimovich, Michal Lichtenstein, Samar Zabit, Sandrine Benhamron, Karameh Akarieh, Kumudesh Mishra, Dinorah Barasch, Ann Saada, Tamar Ziv, Or Kakhlon, Haya Lorberboum-Galski and Hanna Rosenmann
Cells 2023, 12(7), 1006; https://doi.org/10.3390/cells12071006 - 24 Mar 2023
Cited by 8 | Viewed by 3651
Abstract
We recently reported the benefit of the IV transferring of active exogenous mitochondria in a short-term pharmacological AD (Alzheimer’s disease) model. We have now explored the efficacy of mitochondrial transfer in 5XFAD transgenic mice, aiming to explore the underlying mechanism by which the [...] Read more.
We recently reported the benefit of the IV transferring of active exogenous mitochondria in a short-term pharmacological AD (Alzheimer’s disease) model. We have now explored the efficacy of mitochondrial transfer in 5XFAD transgenic mice, aiming to explore the underlying mechanism by which the IV-injected mitochondria affect the diseased brain. Mitochondrial transfer in 5XFAD ameliorated cognitive impairment, amyloid burden, and mitochondrial dysfunction. Exogenously injected mitochondria were detected in the liver but not in the brain. We detected alterations in brain proteome, implicating synapse-related processes, ubiquitination/proteasome-related processes, phagocytosis, and mitochondria-related factors, which may lead to the amelioration of disease. These changes were accompanied by proteome/metabolome alterations in the liver, including pathways of glucose, glutathione, amino acids, biogenic amines, and sphingolipids. Altered liver metabolites were also detected in the serum of the treated mice, particularly metabolites that are known to affect neurodegenerative processes, such as carnosine, putrescine, C24:1-OH sphingomyelin, and amino acids, which serve as neurotransmitters or their precursors. Our results suggest that the beneficial effect of mitochondrial transfer in the 5XFAD mice is mediated by metabolic signaling from the liver via the serum to the brain, where it induces protective effects. The high efficacy of the mitochondrial transfer may offer a novel AD therapy. Full article
(This article belongs to the Special Issue Mitochondrial Dysfunction in Neurological Disorders: Molecular Mechanisms and Potential Points for Intervention)
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20 pages, 2369 KiB  
Article
Tau Transfer via Extracellular Vesicles Disturbs the Astrocytic Mitochondrial System
by Romain Perbet, Valentin Zufferey, Elodie Leroux, Enea Parietti, Jeanne Espourteille, Lucas Culebras, Sylvain Perriot, Renaud Du Pasquier, Séverine Bégard, Vincent Deramecourt, Nicole Déglon, Nicolas Toni, Luc Buée, Morvane Colin and Kevin Richetin
Cells 2023, 12(7), 985; https://doi.org/10.3390/cells12070985 - 23 Mar 2023
Cited by 7 | Viewed by 4291
Abstract
Tauopathies are neurodegenerative disorders involving the accumulation of tau isoforms in cell subpopulations such as astrocytes. The origins of the 3R and 4R isoforms of tau that accumulate in astrocytes remain unclear. Extracellular vesicles (EVs) were isolated from primary neurons overexpressing 1N3R or [...] Read more.
Tauopathies are neurodegenerative disorders involving the accumulation of tau isoforms in cell subpopulations such as astrocytes. The origins of the 3R and 4R isoforms of tau that accumulate in astrocytes remain unclear. Extracellular vesicles (EVs) were isolated from primary neurons overexpressing 1N3R or 1N4R tau or from human brain extracts (progressive supranuclear palsy or Pick disease patients or controls) and characterized (electron microscopy, nanoparticle tracking analysis (NTA), proteomics). After the isolated EVs were added to primary astrocytes or human iPSC-derived astrocytes, tau transfer and mitochondrial system function were evaluated (ELISA, immunofluorescence, MitoTracker staining). We demonstrated that neurons in which 3R or 4R tau accumulated had the capacity to transfer tau to astrocytes and that EVs were essential for the propagation of both isoforms of tau. Treatment with tau-containing EVs disrupted the astrocytic mitochondrial system, altering mitochondrial morphology, dynamics, and redox state. Although similar levels of 3R and 4R tau were transferred, 3R tau-containing EVs were significantly more damaging to astrocytes than 4R tau-containing EVs. Moreover, EVs isolated from the brain fluid of patients with different tauopathies affected mitochondrial function in astrocytes derived from human iPSCs. Our data indicate that tau pathology spreads to surrounding astrocytes via EVs-mediated transfer and modifies their function. Full article
(This article belongs to the Special Issue Mitochondrial Dysfunction in Neurological Disorders: Molecular Mechanisms and Potential Points for Intervention)
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19 pages, 4507 KiB  
Article
Sigma-1 Receptor as a Protective Factor for Diabetes-Associated Cognitive Dysfunction via Regulating Astrocytic Endoplasmic Reticulum-Mitochondrion Contact and Endoplasmic Reticulum Stress
by Mengyu Du, Tao Jiang, Shuxuan He, Bo Cheng, Xin Zhang, Liya Li, Lan Yang, Wei Gao, Yansong Li and Qiang Wang
Cells 2023, 12(1), 197; https://doi.org/10.3390/cells12010197 - 3 Jan 2023
Cited by 4 | Viewed by 3053
Abstract
The prevalence of diabetes-associated cognitive dysfunction (DACD) has increased to 13.5%. Dementia, as the most severe DACD, is the second leading cause of death in patients with diabetes mellitus. Hence, the potential mechanisms of DACD for slowing or halting its progression need to [...] Read more.
The prevalence of diabetes-associated cognitive dysfunction (DACD) has increased to 13.5%. Dementia, as the most severe DACD, is the second leading cause of death in patients with diabetes mellitus. Hence, the potential mechanisms of DACD for slowing or halting its progression need to be urgently explored. Given that the sigma-1 receptor (Sig-1R), a chaperone protein located in the endoplasmic reticulum (ER)-mitochondrion contact membranes to regulate ER stress (ERS), is associated with cognitive outcomes in neurodegenerative diseases, this study aimed to investigate the role of astrocytic Sig-1R in DACD and its underlying mechanism. Here, we examined the levels of ERS and complement component 3/3a (C3/C3a) from primary astrocytes with different concentrations of glucose and treatment. Subsequently, HT22 neurons were cultured in different astrocyte-conditioned medium, and the expression of synaptic proteins was detected. We constructed type 1 diabetes mellitus (T1DM) model to evaluate the astrocytic Sig-1R mechanism on synapse and cognitive function changes. In vitro, high glucose concentration downregulated Sig-1R and aggravated ERS in astrocytes, resulting in synapse deficits. PRE-084, a high-affinity and selective Sig-1R agonist, inhibited astrocytic ERS and complement cascades and restored synaptic damage, while the Sig-1R antagonist displayed the opposite results. Moreover, C3a receptor antagonist (C3aRA) could mimic the effect of PRE-084 and exerted neuroprotective effects. In vivo, PRE-084 substantially reduced ER-mitochondrion contact, activation of ERS, and C3/C3a secretion in mice with T1DM. Additionally, the synaptic loss and neurobehavioral dysfunction of mice with T1DM were less pronounced in both the PRE-084 and C3aRA treatment groups. These findings demonstrated that Sig-1R activation reduced the astrocytic ER-mitochondrion contact, ERS activation, and complement-mediated synaptic damage in T1DM. This study suggested the mechanisms and potential therapeutic approaches for treating DACD. Full article
(This article belongs to the Special Issue Mitochondrial Dysfunction in Neurological Disorders: Molecular Mechanisms and Potential Points for Intervention)
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