Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
4.3
3.2
Journals
Life
Special Issues
Neuroinflammation in Huntington’s Disease: Detrimental Crosstalk
share announcement

Neuroinflammation in Huntington’s Disease: Detrimental Crosstalk

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Physiology and Pathology".

Deadline for manuscript submissions: 30 June 2025 | Viewed by 1955

Special Issue Editors

Dr. Michele D'Angelo

E-Mail Website
Guest Editor
Department of Life, Health and Environmental Sciences, University of L’Aquila, 67100 L'Aquila, Italy
Interests: neurodegeneration; neurodegenerative disorders; neuronal cell rearrangement; Parkinson; PPARs
Special Issues, Collections and Topics in MDPI journals
Dr. Vanessa Castelli

E-Mail
Guest Editor
Department of Life, Health and Environmental Sciences, University of L’Aquila, 67100 L'Aquila, Italy
Interests: neurodegenerative diseases; neurodegeneration; neurological disorders; Parkinson’s disease; probiotics; neuropathic pain; stem cells
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Huntington’s disease (HD) is a devastating condition caused by the repeat expansion of the CAG trinucleotide in exon 1 of the HTT gene, culminating in the production of a mutant huntingtin (mHtt) protein with an extended polyglutamine tract and a toxic gain-of-function. In a number of decades, efforts from researchers across the globe have led to a clear depiction of the consequences of such genetic alteration, paving the way for the development of therapeutic strategies. However, our understanding of the pathophysiology of HD is not complete. Most of all, there is a lack of knowledge as to how the mutated protein spreads across the organism, affecting different cells and tissues in different ways, particularly the brain. In the central nervous system (CNS), mHtt impairs its neuronal physiology by altering molecular systems and the surrounding microenvironment, ultimately causing neuroinflammation. In this context, the involvement of glial cells in the establishment of detrimental pro-inflammatory crosstalk with HD-affected neurons is crucial. Thus, the aim of this Special Issue is to collect studies or review articles describing such crosstalk and the journey of the mHtt protein into the CNS, demonstrating the cellular rearrangements and dynamics that are eventually involved. Moreover, studies or review articles describing the potentiality of restoring anti-inflammatory crosstalk—ultimately from a therapeutic point of view—are most welcomed.

Dr. Michele D'Angelo
Dr. Vanessa Castelli
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. Life 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 2600 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

  • Huntington’s disease
  • mutant huntingtin (mHtt) protein
  • central nervous system
  • mutated protein
  • glial cells

Benefits of Publishing in a Special Issue

  • Ease of navigation: Grouping papers by topic helps scholars navigate broad scope journals more efficiently.
  • Greater discoverability: Special Issues support the reach and impact of scientific research. Articles in Special Issues are more discoverable and cited more frequently.
  • Expansion of research network: Special Issues facilitate connections among authors, fostering scientific collaborations.
  • External promotion: Articles in Special Issues are often promoted through the journal's social media, increasing their visibility.
  • e-Book format: Special Issues with more than 10 articles can be published as dedicated e-books, ensuring wide and rapid dissemination.

Further information on MDPI's Special Issue policies can be found here.

Published Papers (2 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Research

Jump to: Review

14 pages, 1812 KiB  
Article
Evaluation of an Antisense Oligonucleotide Targeting CAG Repeats: A Patient-Customized Therapy Study for Huntington’s Disease
by Sergio Adrian Ocampo-Ortega, Vivany Maydel Sierra-Sanchez, Citlali Margarita Blancas-Napoles, Asdrúbal González-Carteño, Elvia Mera-Jiménez, Martha Edith Macías-Pérez, Adriana Hernandez-Guerra, Rodrigo Romero-Nava, Fengyang Huang, Enrique Hong and Santiago Villafaña
Life 2024, 14(12), 1607; https://doi.org/10.3390/life14121607 - 4 Dec 2024
Viewed by 1237
Abstract
Huntington’s disease is a genetic disorder characterized by progressive neuronal cell damage in some areas of the brain; symptoms are commonly associated with chorea, rigidity and dystonia. The symptoms in Huntington’s Disease are caused by a pathological increase in the number of Cytokine-Adenine-Guanine [...] Read more.
Huntington’s disease is a genetic disorder characterized by progressive neuronal cell damage in some areas of the brain; symptoms are commonly associated with chorea, rigidity and dystonia. The symptoms in Huntington’s Disease are caused by a pathological increase in the number of Cytokine-Adenine-Guanine (CAG) repeats on the first exon of the Huntingtin gene, which causes a protein to have an excessive number of glutamine residues; this alteration leads to a change in the protein’s conformation and function. Therefore, the purpose of this work was to design, synthesize and evaluate an antisense oligonucleotide (ASO; 95 nucleotides) HTT 90-5 directed to the Huntingtin CAG repeats in primary leukocyte culture cells from a patient with Huntington’s Disease; approximately 500,000 leukocytes per well extracted from venous blood were used, to which 100 pMol of ASO were administered, and the expression of Huntingtin was subsequently evaluated at 72 h by RT-PCR. Our results showed that the administration of the HTT 90-5 antisense decreased the expression of Huntingtin mRNA in the primary culture leukocyte cells from our patient. These results suggest that the use of long antisense targeting the CAG Huntingtin cluster may be an option to decrease the expression of Huntingtin and probably could be adjusted depending on the number of CAG repeats in the cluster. Full article
(This article belongs to the Special Issue Neuroinflammation in Huntington’s Disease: Detrimental Crosstalk)
►▼ Show Figures

Figure 1

Review

Jump to: Research

22 pages, 2757 KiB  
Review
Antioxidant and Anti-Inflammatory Defenses in Huntington’s Disease: Roles of NRF2 and PGC-1α, and Therapeutic Strategies
by Francesco D’Egidio, Elvira Qosja, Fabrizio Ammannito, Skender Topi, Michele d’Angelo, Annamaria Cimini and Vanessa Castelli
Life 2025, 15(4), 577; https://doi.org/10.3390/life15040577 - 1 Apr 2025
Viewed by 422
Abstract
Huntington’s disease (HD) is a detrimental neurodegenerative disease caused by the expansion of a CAG triplet in the HTT gene. This mutation leads to the production of mutant Huntingtin (Htt) protein with toxic gain-of-function. The mHtt is responsible in several ways for the [...] Read more.
Huntington’s disease (HD) is a detrimental neurodegenerative disease caused by the expansion of a CAG triplet in the HTT gene. This mutation leads to the production of mutant Huntingtin (Htt) protein with toxic gain-of-function. The mHtt is responsible in several ways for the establishment of an intricate pathogenetic scenario in affected cells, particularly in HD neurons. Among the features of HD, oxidative stress plays a relevant role in the progression of the disease at the cellular level. Mitochondrial dysfunction, bioenergetic deficits, Reactive Oxygen Species (ROS) production, neuroinflammation, and general reduction of antioxidant levels are all involved in the promotion of a toxic oxidative environment, eventually causing cell death. Nonetheless, neuronal cells exert antioxidant molecules to build up defense mechanisms. Key components of these defensive mechanisms are the nuclear factor erythroid 2-related factor 2 (NRF2) and peroxisome proliferator-activated receptor gamma coactivator-1 α (PGC-1α). Thus, this review aims to describe the involvement of oxidative stress in HD by exploring the roles of NRF2 and PGC-1α, crucial actors in this play. Finally, antioxidant therapeutic strategies targeting such markers are discussed. Full article
(This article belongs to the Special Issue Neuroinflammation in Huntington’s Disease: Detrimental Crosstalk)
►▼ Show Figures

Figure 1

Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-2
Back to TopTop