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Recent Advances and Future Prospectives for Experimental Models of Neurodegenerative...
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Recent Advances and Future Prospectives for Experimental Models of Neurodegenerative Diseases

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

Deadline for manuscript submissions: closed (20 March 2021) | Viewed by 16819

Special Issue Editor

Dr. Christina Doriana Orrú

E-Mail Website
Guest Editor
Laboratory of Persistent Viral Diseases (LPVD), Rocky Mountain Laboratories (RML) National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)903 S 4th St, Hamilton, MT 59840, USA
Interests: prion diseases; Parkinson’s disease; tauopathies

Special Issue Information

Neurodegenerative diseases are progressive and incurable. They are associated with the accumulation of a misfolded protein and range from rare conditions, such as Creutzfeldt–Jakob disease (CJD), in which the misfolded protein is the prion protein, to more common disorders such as Alzheimer’s’ and Parkinson’s disease, characterized by Abeta and Tau or Alpha-synuclein brain deposits, respectively.

Recently, much progress has been made in the development of a variety of experimental models to investigate these pathologies, from cellular and tissue/organoid systems to cell-free protein misfolding amplification techniques. This Special Issue aims at highlighting recent advances made using these new platforms to study all aspects of neurodegenerative diseases. 

We encourage researchers interested in these topics to present research articles or reviews that describe the use of cell, tissue, organoid and in vitro protein aggregation assays to study mechanisms of aggregation or clearance, and tissue distribution for these proteins. We welcome reports on model or assay development as well as validation of previously described assays. We are interested in papers that explore co-morbidities and strain discrimination, the use of neurodegenerative disease models to identify therapeutics, the use as surrogate for animal bioassays, as well as more biochemistry oriented mechanistic studies.  

Dr. Christina Doriana Orrú
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. 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

  • Prion diseases
  • Protein misfolding cell-free conversion assays
  • RT-QuIC
  • PMCA
  • ASA
  • Parkinson’s disease
  • Alzheimer’s disease
  • Tauopathies
  • Neurodegenerative diseases

Published Papers (4 papers)

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Research

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17 pages, 2853 KiB  
Article
Central Alteration in Peripheral Neuropathy of Trembler-J Mice: Hippocampal pmp22 Expression and Behavioral Profile in Anxiety Tests
by Juan Pablo Damián, Lucia Vázquez Alberdi, Lucía Canclini, Gonzalo Rosso, Silvia Olivera Bravo, Mariana Martínez, Natalia Uriarte, Paul Ruiz, Miguel Calero, María Vittoria Di Tomaso and Alejandra Kun
Biomolecules 2021, 11(4), 601; https://0-doi-org.brum.beds.ac.uk/10.3390/biom11040601 - 19 Apr 2021
Cited by 7 | Viewed by 2626
Abstract
Charcot–Marie–Tooth (CMT) type 1 disease is the most common human hereditary demyelinating neuropathy. Mutations in pmp22 cause about 70% of all CMT1. Trembler-J (TrJ/+) mice are an animal model of CMT1E, having the same spontaneous pmp22 mutation that is found in humans. We [...] Read more.
Charcot–Marie–Tooth (CMT) type 1 disease is the most common human hereditary demyelinating neuropathy. Mutations in pmp22 cause about 70% of all CMT1. Trembler-J (TrJ/+) mice are an animal model of CMT1E, having the same spontaneous pmp22 mutation that is found in humans. We compared the behavior profile of TrJ/+ and +/+ (wild-type) in open-field and elevated-plus-maze anxiety tests. In these tests, TrJ/+ showed an exclusive head shake movement, a lower frequency of rearing, but a greater frequency of grooming. In elevated-plus-maze, TrJ/+ defecate more frequently, performed fewer total entries, and have fewer entries to closed arms. These hippocampus-associated behaviors in TrJ/+ are consistent with increased anxiety levels. The expression of pmp22 and soluble PMP22 were evaluated in E17-hippocampal neurons and adult hippocampus by in situ hybridization and successive immunohistochemistry. Likewise, the expression of pmp22 was confirmed by RT-qPCR in the entire isolated hippocampi of both genotypes. Moreover, the presence of aggregated PMP22 was evidenced in unmasked granular hippocampal adult neurons and shows genotypic differences. We showed for the first time a behavior profile trait associated with anxiety and a differential expression of pmp22/PMP22 in hippocampal neurons of TrJ/+ and +/+ mice, demonstrating the involvement at the central level in an animal model of peripheral neuropathy (CMT1E). Full article
(This article belongs to the Special Issue Recent Advances and Future Prospectives for Experimental Models of Neurodegenerative Diseases)
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16 pages, 2124 KiB  
Article
Clinical Phenotypes of Parkinson’s Disease Associate with Distinct Gut Microbiota and Metabolome Enterotypes
by Sarah Vascellari, Marta Melis, Vanessa Palmas, Silvia Pisanu, Alessandra Serra, Daniela Perra, Maria Laura Santoru, Valentina Oppo, Roberto Cusano, Paolo Uva, Luigi Atzori, Micaela Morelli, Giovanni Cossu and Aldo Manzin
Biomolecules 2021, 11(2), 144; https://0-doi-org.brum.beds.ac.uk/10.3390/biom11020144 - 22 Jan 2021
Cited by 33 | Viewed by 4676
Abstract
Parkinson’s disease (PD) is a clinically heterogenic disorder characterized by distinct clinical entities. Most studies on motor deficits dichotomize PD into tremor dominant (TD) or non-tremor dominant (non-TD) with akinetic-rigid features (AR). Different pathophysiological mechanisms may affect the onset of motor manifestations. Recent [...] Read more.
Parkinson’s disease (PD) is a clinically heterogenic disorder characterized by distinct clinical entities. Most studies on motor deficits dichotomize PD into tremor dominant (TD) or non-tremor dominant (non-TD) with akinetic-rigid features (AR). Different pathophysiological mechanisms may affect the onset of motor manifestations. Recent studies have suggested that gut microbes may be involved in PD pathogenesis. The aim of this study was to investigate the gut microbiota and metabolome composition in PD patients in relation to TD and non-TD phenotypes. In order to address this issue, gut microbiota and the metabolome structure of PD patients were determined from faecal samples using 16S next generation sequencing and gas chromatography–mass spectrometry approaches. The results showed a reduction in the relative abundance of Lachnospiraceae, Blautia, Coprococcus, Lachnospira, and an increase in Enterobacteriaceae, Escherichia and Serratia linked to non-TD subtypes. Moreover, the levels of important molecules (i.e., nicotinic acid, cadaverine, glucuronic acid) were altered in relation to the severity of phenotype. We hypothesize that the microbiota/metabolome enterotypes associated to non-TD subtypes may favor the development of gut inflammatory environment and gastrointestinal dysfunctions and therefore a more severe α-synucleinopathy. This study adds important information to PD pathogenesis and emphasizes the potential pathophysiological link between gut microbiota/metabolites and PD motor subtypes. Full article
(This article belongs to the Special Issue Recent Advances and Future Prospectives for Experimental Models of Neurodegenerative Diseases)
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Review

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24 pages, 980 KiB  
Review
From Cell Culture to Organoids-Model Systems for Investigating Prion Strain Characteristics
by Hailey Pineau and Valerie L. Sim
Biomolecules 2021, 11(1), 106; https://doi.org/10.3390/biom11010106 - 14 Jan 2021
Cited by 10 | Viewed by 4029
Abstract
Prion diseases are the hallmark protein folding neurodegenerative disease. Their transmissible nature has allowed for the development of many different cellular models of disease where prion propagation and sometimes pathology can be induced. This review examines the range of simple cell cultures to [...] Read more.
Prion diseases are the hallmark protein folding neurodegenerative disease. Their transmissible nature has allowed for the development of many different cellular models of disease where prion propagation and sometimes pathology can be induced. This review examines the range of simple cell cultures to more complex neurospheres, organoid, and organotypic slice cultures that have been used to study prion disease pathogenesis and to test therapeutics. We highlight the advantages and disadvantages of each system, giving special consideration to the importance of strains when choosing a model and when interpreting results, as not all systems propagate all strains, and in some cases, the technique used, or treatment applied, can alter the very strain properties being studied. Full article
(This article belongs to the Special Issue Recent Advances and Future Prospectives for Experimental Models of Neurodegenerative Diseases)
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27 pages, 3565 KiB  
Review
Insights into the Conserved Regulatory Mechanisms of Human and Yeast Aging
by Rashmi Dahiya, Taj Mohammad, Mohamed F. Alajmi, Md. Tabish Rehman, Gulam Mustafa Hasan, Afzal Hussain and Md. Imtaiyaz Hassan
Biomolecules 2020, 10(6), 882; https://0-doi-org.brum.beds.ac.uk/10.3390/biom10060882 - 09 Jun 2020
Cited by 17 | Viewed by 4604
Abstract
Aging represents a significant biological process having strong associations with cancer, diabetes, and neurodegenerative and cardiovascular disorders, which leads to progressive loss of cellular functions and viability. Astonishingly, age-related disorders share several genetic and molecular mechanisms with the normal aging process. Over the [...] Read more.
Aging represents a significant biological process having strong associations with cancer, diabetes, and neurodegenerative and cardiovascular disorders, which leads to progressive loss of cellular functions and viability. Astonishingly, age-related disorders share several genetic and molecular mechanisms with the normal aging process. Over the last three decades, budding yeast Saccharomyces cerevisiae has emerged as a powerful yet simple model organism for aging research. Genetic approaches using yeast RLS have led to the identification of hundreds of genes impacting lifespan in higher eukaryotes. Numerous interventions to extend yeast lifespan showed an analogous outcome in multi-cellular eukaryotes like fruit flies, nematodes, rodents, and humans. We collected and analyzed a multitude of observations from published literature and provide the contribution of yeast in the understanding of aging hallmarks most applicable to humans. Here, we discuss key pathways and molecular mechanisms that underpin the evolutionarily conserved aging process and summarize the current understanding and clinical applicability of its trajectories. Gathering critical information on aging biology would pave the way for future investigation targeted at the discovery of aging interventions. Full article
(This article belongs to the Special Issue Recent Advances and Future Prospectives for Experimental Models of Neurodegenerative Diseases)
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