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Cells
Special Issues
Mitochondria and Other Organelles in Neurodegenerative Diseases
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Mitochondria and Other Organelles in Neurodegenerative Diseases

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Intracellular and Plasma Membranes".

Deadline for manuscript submissions: 31 January 2025 | Viewed by 3629

Special Issue Editor

Dr. Tian Liu

E-Mail Website
Guest Editor
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA
Interests: mitochondrial dysfunction; MAM; mitophagy; tauopathy; TDP-43 pathology; Alzheimer’s disease; FTD-ALS; neurodegeneration

Special Issue Information

Dear Colleagues,

The majority of neurodegenerative disorders have been linked to mitochondrial dysfunction and its associated organelles. These essential components play a vital role in maintaining cellular homeostasis, ensuring neuronal health and proper neuronal function. In the early stages of neurodegenerative diseases, including Alzheimer's, Parkinson's, and Huntington's disease, as well as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), consistent signs of mitochondrial dysfunction emerge. Nevertheless, the precise mechanisms underlying these dysfunctions have yet to be elucidated, and additional therapeutic approaches must be investigated. This Special Issue of Cells solicits original research articles and reviews that contribute to our understanding of the molecular and cellular mechanisms underlying neurodegenerative diseases and therapeutic strategies.

Dr. Tian Liu
Guest Editor

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. Cells is an international peer-reviewed open access semimonthly 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 dysfunction
  • ER stress
  • TDP-43 pathology
  • protein aggregation
  • tauopathy
  • neuroinflammation
  • neurodegeneration
  • neurodegenerative diseases
  • therapeutic approaches

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Published Papers (2 papers)

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Research

15 pages, 5244 KiB  
Communication
APOE4 Increases Energy Metabolism in APOE-Isogenic iPSC-Derived Neurons
by Vanessa Budny, Yannic Knöpfli, Debora Meier, Kathrin Zürcher, Chantal Bodenmann, Siri L. Peter, Terry Müller, Marie Tardy, Cedric Cortijo and Christian Tackenberg
Cells 2024, 13(14), 1207; https://doi.org/10.3390/cells13141207 - 17 Jul 2024
Cited by 1 | Viewed by 1327
Abstract
The apolipoprotein E4 (APOE4) allele represents the major genetic risk factor for Alzheimer’s disease (AD). In contrast, APOE2 is known to lower the AD risk, while APOE3 is defined as risk neutral. APOE plays a prominent role in the bioenergetic homeostasis [...] Read more.
The apolipoprotein E4 (APOE4) allele represents the major genetic risk factor for Alzheimer’s disease (AD). In contrast, APOE2 is known to lower the AD risk, while APOE3 is defined as risk neutral. APOE plays a prominent role in the bioenergetic homeostasis of the brain, and early-stage metabolic changes have been detected in the brains of AD patients. Although APOE is primarily expressed by astrocytes in the brain, neurons have also been shown as source for APOE. However, the distinct roles of the three APOE isoforms in neuronal energy homeostasis remain poorly understood. In this study, we generated pure human neurons (iN cells) from APOE-isogenic induced pluripotent stem cells (iPSCs), expressing either APOE2, APOE3, APOE4, or carrying an APOE knockout (KO) to investigate APOE isoform-specific effects on neuronal energy metabolism. We showed that endogenously produced APOE4 enhanced mitochondrial ATP production in APOE-isogenic iN cells but not in the corresponding iPS cell line. This effect neither correlated with the expression levels of mitochondrial fission or fusion proteins nor with the intracellular or secreted levels of APOE, which were similar for APOE2, APOE3, and APOE4 iN cells. ATP production and basal respiration in APOE-KO iN cells strongly differed from APOE4 and more closely resembled APOE2 and APOE3 iN cells, indicating a gain-of-function mechanism of APOE4 rather than a loss-of-function. Taken together, our findings in APOE isogenic iN cells reveal an APOE genotype-dependent and neuron-specific regulation of oxidative energy metabolism. Full article
(This article belongs to the Special Issue Mitochondria and Other Organelles in Neurodegenerative Diseases)
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18 pages, 3602 KiB  
Article
Disruption of Mitophagy Flux through the PARL-PINK1 Pathway by CHCHD10 Mutations or CHCHD10 Depletion
by Tian Liu, Liam Wetzel, Zexi Zhu, Pavan Kumaraguru, Viraj Gorthi, Yan Yan, Mohammed Zaheen Bukhari, Aizara Ermekbaeva, Hanna Jeon, Teresa R. Kee, Jung-A Alexa Woo and David E. Kang
Cells 2023, 12(24), 2781; https://doi.org/10.3390/cells12242781 - 7 Dec 2023
Cited by 2 | Viewed by 1872
Abstract
Coiled-coil-helix-coiled-coil-helix domain-containing 10 (CHCHD10) is a nuclear-encoded mitochondrial protein which is primarily mutated in the spectrum of familial and sporadic amyotrophic lateral sclerosis (ALS)–frontotemporal dementia (FTD). Endogenous CHCHD10 levels decline in the brains of ALS–FTD patients, and the CHCHD10S59L mutation in Drosophila [...] Read more.
Coiled-coil-helix-coiled-coil-helix domain-containing 10 (CHCHD10) is a nuclear-encoded mitochondrial protein which is primarily mutated in the spectrum of familial and sporadic amyotrophic lateral sclerosis (ALS)–frontotemporal dementia (FTD). Endogenous CHCHD10 levels decline in the brains of ALS–FTD patients, and the CHCHD10S59L mutation in Drosophila induces dominant toxicity together with PTEN-induced kinase 1 (PINK1), a protein critical for the induction of mitophagy. However, whether and how CHCHD10 variants regulate mitophagy flux in the mammalian brain is unknown. Here, we demonstrate through in vivo and in vitro models, as well as human FTD brain tissue, that ALS/FTD-linked CHCHD10 mutations (R15L and S59L) impair mitophagy flux and mitochondrial Parkin recruitment, whereas wild-type CHCHD10 (CHCHD10WT) normally enhances these measures. Specifically, we show that CHCHD10R15L and CHCHD10S59L mutations reduce PINK1 levels by increasing PARL activity, whereas CHCHD10WT produces the opposite results through its stronger interaction with PARL, suppressing its activity. Importantly, we also demonstrate that FTD brains with TAR DNA-binding protein-43 (TDP-43) pathology demonstrate disruption of the PARL–PINK1 pathway and that experimentally impairing mitophagy promotes TDP-43 aggregation. Thus, we provide herein new insights into the regulation of mitophagy and TDP-43 aggregation in the mammalian brain through the CHCHD10–PARL–PINK1 pathway. Full article
(This article belongs to the Special Issue Mitochondria and Other Organelles in Neurodegenerative Diseases)
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