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Metabolites
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Cellular Metabolism in Neurological Disorders
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Cellular Metabolism in Neurological Disorders

A special issue of Metabolites (ISSN 2218-1989). This special issue belongs to the section "Cell Metabolism".

Deadline for manuscript submissions: 30 November 2024 | Viewed by 10078

Special Issue Editors

Dr. Wenting Guo

E-Mail Website
Guest Editor
1. Lab of Neurobiology, VIB-KU Leuven Center for Brain & Disease Research, O&N5, Box 602, Campus Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium
2. KU Leuven- Stem Cell Institute (SCIL), KU Leuven University, ON4 Herestraat 49 - Box 804, Campus Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium
Interests: amyotrophic lateral sclerosis; frontotemporal Dementia; Charcot–Marie–Tooth disease; regenerative medicine; neurodegeneration; CRISPR-Cas9; translational research
Dr. Laura Fumagalli

E-Mail Website
Guest Editor
Microglia and Inflammation in Neurological Disorders (MIND) Lab, VIB-Center for Molecular Neurology (CMN), VIB. Universiteit Antwerpen-CDE, Parking P4, Gebouw V 0.10 Universiteitsplein 1, B-2610 Antwerpen, Belgium
Interests: neurodegeneration; amyotrophic lateral sclerosis; frontotemporal dementia; C9orf72; axonal transport; neuroinflammation
Dr. Teodoro Bottiglieri

E-Mail Website
Guest Editor
Scott and White, Center of Metabolomics, Temple, TX 76712, USA
Interests: homocysteine metabolism; folate; vitamin B12 and one-carbon metabolism; regulation of S-adenosylmethionine and methylation in metabolic disorders; amino acid metabolism energy metabolism in the central nervous system; inherited disorders affecting neurotransmitter metabolism; risk factors for vascular and neurodegenerative diseases
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Recent years have witnessed a significant growth of evidence that show dysregulated energy metabolism in neurological disorders. Neurons are large, polarized, excitable cells and, therefore, face unique challenges to maintain energy homeostasis. As such, they are the main contributors to the huge energy demand of the central nervous system (CNS). The remarkable vulnerability of neuronal cells to energy reduction contributes to disease susceptibility and progression. In addition, mitochondrial dysfunction has been widely recognized as a typical clinical hallmark of several neurodegenerative disorders, including Huntington's disease, Parkinson's disease, Amyotrophic lateral sclerosis, Epilepsy, Schizophrenia, Multiple sclerosis, Neuropathic pain, and Alzheimer's disease. As the energy supply center of cells, the function of mitochondria has been extensively investigated in relation to the metabolism feature of CNS. Furthermore, glycogen metabolism has also been an important implication for the functioning of the brain, especially for the cooperation between astrocytes and neurons. In this Special Issue, we welcome any original research articles, short reports, reviews, and case reports that explore the impact and mechanisms of metabolic alterations in neurological disorders and provide a forum to discuss emerging metabolism-centric therapeutic avenues.

Dr. Wenting Guo
Dr. Laura Fumagalli
Dr. Teodoro Bottiglieri
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. Metabolites 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

  • metabolism 
  • neurodegeneration 
  • mitochondria 
  • glycogen
  • therapeutic targets
  • neurons
  • glia

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Published Papers (4 papers)

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Research

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11 pages, 517 KiB  
Article
Peripheral Brain-Derived Neurotrophic Factor (BDNF) and Its Regulatory miRNAs as Biological Correlates of Impulsivity in Young Adults
by Przemyslaw Zakowicz, Beata Narozna, Tomasz Kozlowski, Weronika Bargiel, Maksymilian Grabarczyk, Maria Terczynska, Julia Pilecka, Karolina Wasicka-Przewozna, Joanna Pawlak and Maria Skibinska
Metabolites 2024, 14(10), 529; https://doi.org/10.3390/metabo14100529 - 30 Sep 2024
Viewed by 788
Abstract
Background: Impulsivity assessment may serve as a valuable clinical tool in the stratification of suicide risk. Acting without forethought is a crucial feature in the psychopathology of many psychiatric disturbances and corresponds with suicidal ideations, behaviors, and attempts. Methods: We present [...] Read more.
Background: Impulsivity assessment may serve as a valuable clinical tool in the stratification of suicide risk. Acting without forethought is a crucial feature in the psychopathology of many psychiatric disturbances and corresponds with suicidal ideations, behaviors, and attempts. Methods: We present data on biological and psychological correlates of impulsivity among young adults (n = 47). Psychological analysis included both the self-description questionnaire—Barratt Impulsiveness Scale (BIS-11)—and neuropsychological behavioral tests, including the Iowa Gambling Task (IGT), the Simple Response Time task (SRT), and the Continuous Performance Test (CPT). mRNA and micro-RNA were isolated from peripheral blood mononuclear cells (PBMC). Expression levels of Brain-Derived Neurotrophic Factor (BDNF) mRNA and its regulatory micro RNAs, mir-1-3p, mir-15a-5p, mir-26a-5p, mir-26b-5p, and mir-195-5p, were analyzed using the quantitative reverse transcription polymerase chain reaction (RT-qPCR) method. proBDNF and BDNF plasma protein levels were quantified using enzyme-linked immunosorbent assay (ELISA). Results: Significant correlations between BDNF mRNA and mir-15a-5p as well as proBDNF levels and mir-1-3p were detected. proBDNF protein levels correlated with motor and perseverance, while mir-26b correlated with cognitive complexity subdimensions of the BIS-11 scale. Correlations between BDNF, miRNAs, and the results of neuropsychological tests were also detected. Conclusions: The BDNF pathway shows a clinical potential in searching for biomarkers of impulse-control impairment. BDNF-regulatory micro-RNAs are detectable and related to clinical parameters in the studied population, which needs further research. Full article
(This article belongs to the Special Issue Cellular Metabolism in Neurological Disorders)
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15 pages, 6500 KiB  
Article
Alteration of Autophagy and Glial Activity in Nilotinib-Treated Huntington’s Disease Patients
by Karen E. Anderson, Max Stevenson, Rency Varghese, Michaeline L. Hebron, Erin Koppel, Mara McCartin, Robin Kuprewicz, Sara Matar, Dalila Ferrante and Charbel Moussa
Metabolites 2022, 12(12), 1225; https://doi.org/10.3390/metabo12121225 - 6 Dec 2022
Cited by 5 | Viewed by 2055
Abstract
Nilotinib is a tyrosine kinase inhibitor that is safe and tolerated in neurodegeneration, it achieves CSF concentration that is adequate to inhibit discoidin domain receptor (DDR)-1. Nilotinib significantly affects dopamine metabolites, including Homovanillic acid (HVA), resulting in an increase in brain dopamine. HD [...] Read more.
Nilotinib is a tyrosine kinase inhibitor that is safe and tolerated in neurodegeneration, it achieves CSF concentration that is adequate to inhibit discoidin domain receptor (DDR)-1. Nilotinib significantly affects dopamine metabolites, including Homovanillic acid (HVA), resulting in an increase in brain dopamine. HD is a hereditary disease caused by mutations in the Huntingtin’s (HTT) gene and characterized by neurodegeneration and motor and behavioral symptoms that are associated with activation of dopamine receptors. We explored the effects of a low dose of nilotinib (150 mg) on behavioral changes and motor symptoms in manifest HD patients and examined the effects of nilotinib on several brain mechanisms, including dopamine transmission and gene expression via cerebrospinal fluid (CSF) miRNA sequencing. Nilotinib, 150 mg, did not result in any behavioral changes, although it significantly attenuated HVA levels, suggesting reduction of dopamine catabolism. There was no significant change in HTT, phosphorylated neuro-filament and inflammatory markers in the CSF and plasma via immunoassays. Whole miRNA genome sequencing of the CSF revealed significant longitudinal changes in miRNAs that control specific genes associated with autophagy, inflammation, microglial activity and basal ganglia neurotransmitters, including dopamine and serotonin. Full article
(This article belongs to the Special Issue Cellular Metabolism in Neurological Disorders)
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16 pages, 2175 KiB  
Article
Metabolites of De Novo Purine Synthesis: Metabolic Regulators and Cytotoxic Compounds
by Olga Souckova, Vaclava Skopova, Veronika Baresova, David Sedlak, Anthony J. Bleyer, Stanislav Kmoch and Marie Zikanova
Metabolites 2022, 12(12), 1210; https://doi.org/10.3390/metabo12121210 - 2 Dec 2022
Cited by 2 | Viewed by 4776
Abstract
Cytotoxicity of de novo purine synthesis (DNPS) metabolites is critical to the pathogenesis of three known and one putative autosomal recessive disorder affecting DNPS. These rare disorders are caused by biallelic mutations in the DNPS genes phosphoribosylformylglycineamidine synthase (PFAS), phosphoribosylaminoimidazolecarboxylase/phosphoribosylaminoimidazolesuccinocarboxamide synthase (PAICS), adenylosuccinate [...] Read more.
Cytotoxicity of de novo purine synthesis (DNPS) metabolites is critical to the pathogenesis of three known and one putative autosomal recessive disorder affecting DNPS. These rare disorders are caused by biallelic mutations in the DNPS genes phosphoribosylformylglycineamidine synthase (PFAS), phosphoribosylaminoimidazolecarboxylase/phosphoribosylaminoimidazolesuccinocarboxamide synthase (PAICS), adenylosuccinate lyase (ADSL), and aminoimidazole carboxamide ribonucleotide transformylase/inosine monophosphate cyclohydrolase (ATIC) and are clinically characterized by developmental abnormalities, psychomotor retardation, and nonspecific neurological impairment. At a biochemical level, loss of function of specific mutated enzymes results in elevated levels of DNPS ribosides in body fluids. The main pathogenic effect is attributed to the accumulation of DNPS ribosides, which are postulated to be toxic to the organism. Therefore, we decided to characterize the uptake and flux of several DNPS metabolites in HeLa cells and the impact of DNPS metabolites to viability of cancer cell lines and primary skin fibroblasts. We treated cells with DNPS metabolites and followed their flux in purine synthesis and degradation. In this study, we show for the first time the transport of formylglycinamide ribotide (FGAR), aminoimidazole ribotide (AIR), succinylaminoimidazolecarboxamide ribotide (SAICAR), and aminoimidazolecarboxamide ribotide (AICAR) into cells and their flux in DNPS and the degradation pathway. We found diminished cell viability mostly in the presence of FGAR and AIR. Our results suggest that direct cellular toxicity of DNPS metabolites may not be the primary pathogenetic mechanism in these disorders. Full article
(This article belongs to the Special Issue Cellular Metabolism in Neurological Disorders)
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Review

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25 pages, 751 KiB  
Review
Altered Metabolic Signaling and Potential Therapies in Polyglutamine Diseases
by Alisha Vohra, Patrick Keefe and Prasanth Puthanveetil
Metabolites 2024, 14(6), 320; https://doi.org/10.3390/metabo14060320 - 31 May 2024
Viewed by 1006
Abstract
Polyglutamine diseases comprise a cluster of genetic disorders involving neurodegeneration and movement disabilities. In polyglutamine diseases, the target proteins become aberrated due to polyglutamine repeat formation. These aberrant proteins form the root cause of associated complications. The metabolic regulation during polyglutamine diseases is [...] Read more.
Polyglutamine diseases comprise a cluster of genetic disorders involving neurodegeneration and movement disabilities. In polyglutamine diseases, the target proteins become aberrated due to polyglutamine repeat formation. These aberrant proteins form the root cause of associated complications. The metabolic regulation during polyglutamine diseases is not well studied and needs more attention. We have brought to light the significance of regulating glutamine metabolism during polyglutamine diseases, which could help in decreasing the neuronal damage associated with excess glutamate and nucleotide generation. Most polyglutamine diseases are accompanied by symptoms that occur due to excess glutamate and nucleotide accumulation. Along with a dysregulated glutamine metabolism, the Nicotinamide adenine dinucleotide (NAD+) levels drop down, and, under these conditions, NAD+ supplementation is the only achievable strategy. NAD+ is a major co-factor in the glutamine metabolic pathway, and it helps in maintaining neuronal homeostasis. Thus, strategies to decrease excess glutamate and nucleotide generation, as well as channelizing glutamine toward the generation of ATP and the maintenance of NAD+ homeostasis, could aid in neuronal health. Along with understanding the metabolic dysregulation that occurs during polyglutamine diseases, we have also focused on potential therapeutic strategies that could provide direct benefits or could restore metabolic homeostasis. Our review will shed light into unique metabolic causes and into ideal therapeutic strategies for treating complications associated with polyglutamine diseases. Full article
(This article belongs to the Special Issue Cellular Metabolism in Neurological Disorders)
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