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Molecular Mechanisms and Treatments in Neurodegenerative Diseases
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Molecular Mechanisms and Treatments in Neurodegenerative Diseases

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: 20 December 2024 | Viewed by 550

Special Issue Editors

Dr. Ermanna Turano

E-Mail
Guest Editor
Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, 37134 Verona, Italy
Interests: mesenchymal stem cells; extracellular vesicles; neurodegenerative diseases; regenerative therapy; cell biology; neuroinflammation
Dr. Raffaella Mariotti

E-Mail Website
Guest Editor
Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, 37134 Verona, Italy
Interests: amyotrophic lateral sclerosis; mesenchymal stem cell; extracellular vesicles; neurological diseases

Special Issue Information

Dear Colleagues,

Neurodegenerative diseases, such as Alzheimer’s disease (AD), Parkinson’s disease (PD), and amyotrophic lateral sclerosis (ALS), are characterized by the accumulation of specific proteins within the nervous system accompanied by a progressive loss of neurons in the affected regions. Although some relevant neurodegenerative mechanisms have been identified, such as protein aggregation, oxidative stress, mitochondrial dysfunction, and neuroinflammation, their pathogenic mechanisms are still not fully understood, and the failure to identify the precise causes of neurodegeneration leads to the absence of treatments.

This Special Issue aims to collect basic and preclinical studies on complex pathogenetic mechanisms, focusing on the identification of effective therapeutic strategies (both pharmacological and cellular) to counteract neurodegeneration and potential candidate biomarkers or therapeutic targets.

We welcome original research articles and reviews to discuss disease pathogenesis and new therapeutic strategies to counteract or alleviate neurodegenerative diseases.

Dr. Ermanna Turano
Dr. Raffaella Mariotti
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. 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
  • regenerative therapy
  • neuroprotection
  • neurodegeneration
  • pathogenetic mechanisms
  • cell therapy

Published Papers (1 paper)

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Research

16 pages, 5952 KiB  
Article
Gastrodin Improves the Activity of the Ubiquitin–Proteasome System and the Autophagy–Lysosome Pathway to Degrade Mutant Huntingtin
by He Sun, Miao Li, Yunling Li, Na Zheng, Jiaxin Li, Xiang Li, Yingying Liu, Qianyun Ji, Liping Zhou, Jingwen Su, Wanxu Huang, Zhongbo Liu, Peng Liu and Libo Zou
Int. J. Mol. Sci. 2024, 25(14), 7709; https://doi.org/10.3390/ijms25147709 (registering DOI) - 14 Jul 2024
Viewed by 154
Abstract
Gastrodin (GAS) is the main chemical component of the traditional Chinese herb Gastrodia elata (called “Tianma” in Chinese), which has been used to treat neurological conditions, including headaches, epilepsy, stroke, and memory loss. To our knowledge, it is unclear whether GAS has a [...] Read more.
Gastrodin (GAS) is the main chemical component of the traditional Chinese herb Gastrodia elata (called “Tianma” in Chinese), which has been used to treat neurological conditions, including headaches, epilepsy, stroke, and memory loss. To our knowledge, it is unclear whether GAS has a therapeutic effect on Huntington’s disease (HD). In the present study, we evaluated the effect of GAS on the degradation of mutant huntingtin protein (mHtt) by using PC12 cells transfected with N-terminal mHtt Q74. We found that 0.1–100 μM GAS had no effect on the survival rate of Q23 and Q74 PC12 cells after 24–48 h of incubation. The ubiquitin–proteasome system (UPS) is the main system that clears misfolded proteins in eukaryotic cells. Mutated Htt significantly upregulated total ubiquitinated protein (Ub) expression, decreased chymotrypsin-like, trypsin-like and caspase-like peptidase activity, and reduced the colocalization of the 20S proteasome with mHtt. GAS (25 μM) attenuated all of the abovementioned pathological changes, and the regulatory effect of GAS on mHtt was found to be abolished by MG132, a proteasome inhibitor. The autophagy–lysosome pathway (ALP) is another system for misfolded protein degradation. Although GAS downregulated the expression of autophagy markers (LC3II and P62), it increased the colocalization of LC3II with lysosomal associated membrane protein 1 (LAMP1), which indicates that ALP was activated. Moreover, GAS prevented mHtt-induced neuronal damage in PC12 cells. GAS has a selective effect on mHtt in Q74 PC12 cells and has no effect on Q23 and proteins encoded by other genes containing long CAGs, such as Rbm33 (10 CAG repeats) and Hcn1 (>30 CAG repeats). Furthermore, oral administration of 100 mg/kg GAS increased grip strength and attenuated mHtt aggregates in B6-hHTT130-N transgenic mice. This is a high dose (100 mg/kg GAS) when compared with experiments on HD mice with other small molecules. We will design more doses to evaluate the dose–response relationship of the inhibition effect of GAS on mHtt in our next study. In summary, GAS can promote the degradation of mHtt by activating the UPS and ALP, making it a potential therapeutic agent for HD. Full article
(This article belongs to the Special Issue Molecular Mechanisms and Treatments in Neurodegenerative Diseases)
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