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Special Issue "Stem Cells in Neurodegenerative Pathologies"

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Neurobiology".

Deadline for manuscript submissions: 31 July 2021.

Special Issue Editors

Dr. Šárka Lehtonen
E-Mail Website
Guest Editor
University of Eastern Finland, A.I.Virtanen Institute for Molecular Sciences, Kuopio, Finland Neuroscience Center, HiLIFE, University of Helsinki, Helsinki, Finland
Interests: models of neurodegenerative diseases; schizophrenia and psychopathy; use of stem cells in neurodegeneration and neurodevelopment; blood-brain barrier; organ-on-chip; humanized models; identification of potential therapeutic molecules for brain diseases
Prof. Dr. Amalia Dolga
E-Mail Website
Co-Guest Editor
Department of Molecular Pharmacology, University of Groningen, Groningen, The Netherlands
Interests: neurodegeneration; mitochondrial function; inflammation; potassium channels
Special Issues and Collections in MDPI journals

Special Issue Information

Dear Colleagues,

Neurodegenerative diseases are characterized by the chronic and progressive loss of neuronal functions in specific brain areas, resulting in memory deficit, cognitive impairment, or impaired motor coordination. There is a wide range of hereditary and sporadic neurologic disorders such as Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, and amyotrophic lateral sclerosis. All these diseases occur as a result of neurodegeneration and include typical protein misfolding and aggregation. Although intensive efforts have been made to find a cure, effective treatments for neurodegenerative diseases have not yet been discovered. Possible reasons for this include the lack of appropriate human disease models and a limited understanding of the etiological and neurobiological mechanisms of these conditions.  Experimental models that accurately mimic the development of neurodegenerative diseases in humans are desperately needed to understand the cause and mechanisms of these diseases, as well as to identify new therapeutic targets. Recent advances in stem cell technology have opened the path to the generation of induced pluripotent stem cells (iPSCs) from somatic cells, thus offering an unlimited source of patient-specific disease-relevant cells for more accurate modeling.  In this Special Issue, we aim to summarize the current knowledge in this important field and give researchers the possibility of sharing novel data on the multiple aspects of modeling neurodegeneration by using stem cells in different disease pathologies. We invite experts to contribute with critical reviews and research papers on, but not limited to, the neurodegenerative diseases mentioned above, describing different models and mechanisms that play a role in neurodegeneration and the use of such models in drug discovery.   

Dr. Šárka Lehtonen
Guest Editor
Dr. Amalia Dolga
Co-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 papers will be 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. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. 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

  • neurodegenerative diseases
  • stem cells
  • cell-autonomous and non-cell-autonomous mechanism
  • protein misfolding and aggregation
  • inflammation
  • humanized models
  • drug discoveries

Published Papers (2 papers)

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Research

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Open AccessArticle
Transplantation of Neural Precursors Derived from Induced Pluripotent Cells Preserve Perineuronal Nets and Stimulate Neural Plasticity in ALS Rats
Int. J. Mol. Sci. 2020, 21(24), 9593; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21249593 - 16 Dec 2020
Cited by 1 | Viewed by 789
Abstract
A promising therapeutic strategy for amyotrophic lateral sclerosis (ALS) treatment is stem cell therapy. Neural progenitors derived from induced pluripotent cells (NP-iPS) might rescue or replace dying motoneurons (MNs). However, the mechanisms responsible for the beneficial effect are not fully understood. The aim [...] Read more.
A promising therapeutic strategy for amyotrophic lateral sclerosis (ALS) treatment is stem cell therapy. Neural progenitors derived from induced pluripotent cells (NP-iPS) might rescue or replace dying motoneurons (MNs). However, the mechanisms responsible for the beneficial effect are not fully understood. The aim here was to investigate the mechanism by studying the effect of intraspinally injected NP-iPS into asymptomatic and early symptomatic superoxide dismutase (SOD)1G93A transgenic rats. Prior to transplantation, NP-iPS were characterized in vitro for their ability to differentiate into a neuronal phenotype. Motor functions were tested in all animals, and the tissue was analyzed by immunohistochemistry, qPCR, and Western blot. NP-iPS transplantation significantly preserved MNs, slowed disease progression, and extended the survival of all treated animals. The dysregulation of spinal chondroitin sulfate proteoglycans was observed in SOD1G93A rats at the terminal stage. NP-iPS application led to normalized host genes expression (versican, has-1, tenascin-R, ngf, igf-1, bdnf, bax, bcl-2, and casp-3) and the protection of perineuronal nets around the preserved MNs. In the host spinal cord, transplanted cells remained as progenitors, many in contact with MNs, but they did not differentiate. The findings suggest that NP-iPS demonstrate neuroprotective properties by regulating local gene expression and regulate plasticity by modulating the central nervous system (CNS) extracellular matrix such as perineuronal nets (PNNs). Full article
(This article belongs to the Special Issue Stem Cells in Neurodegenerative Pathologies)
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Review

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Open AccessReview
Utilising Induced Pluripotent Stem Cells in Neurodegenerative Disease Research: Focus on Glia
Int. J. Mol. Sci. 2021, 22(9), 4334; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22094334 - 21 Apr 2021
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Abstract
Induced pluripotent stem cells (iPSCs) are a self-renewable pool of cells derived from an organism’s somatic cells. These can then be programmed to other cell types, including neurons. Use of iPSCs in research has been two-fold as they have been used for human [...] Read more.
Induced pluripotent stem cells (iPSCs) are a self-renewable pool of cells derived from an organism’s somatic cells. These can then be programmed to other cell types, including neurons. Use of iPSCs in research has been two-fold as they have been used for human disease modelling as well as for the possibility to generate new therapies. Particularly in complex human diseases, such as neurodegenerative diseases, iPSCs can give advantages over traditional animal models in that they more accurately represent the human genome. Additionally, patient-derived cells can be modified using gene editing technology and further transplanted to the brain. Glial cells have recently become important avenues of research in the field of neurodegenerative diseases, for example, in Alzheimer’s disease and Parkinson’s disease. This review focuses on using glial cells (astrocytes, microglia, and oligodendrocytes) derived from human iPSCs in order to give a better understanding of how these cells contribute to neurodegenerative disease pathology. Using glia iPSCs in in vitro cell culture, cerebral organoids, and intracranial transplantation may give us future insight into both more accurate models and disease-modifying therapies. Full article
(This article belongs to the Special Issue Stem Cells in Neurodegenerative Pathologies)
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