Piavchenko, Gennadii A., Pokidova, Ksenia S., Kuzmin, Egor A., Venediktov, Artem A., Izmailov, Ilya Y., Meglinski, Igor V. and Kuznetsov, Sergey L. (2024). Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models. Applied Sciences, 14 (24),
Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, often linked to mutations in the FUS gene, leading to toxic protein aggregates. This study investigates the role of HSP70, a molecular chaperone, in mitigating neurodegeneration in FUS-ALS mouse models. Using quantitative immunofluorescence microscopy, we mapped cellular changes in the primary motor cortex of double transgenic FUS/HSP70 mice and compared them to single FUS-transgenic controls. Our results reveal that double transgenic mice exhibit significantly reduced neuronal damage and increased levels of mature neuronal (NeuN) and microglial (Iba1) markers, indicating a protective effect of HSP70. Intracellular HSP70 expression proved more effective than extracellular release, suggesting that targeted HSP70 delivery to neurons may offer a promising therapeutic avenue for ALS. This study underscores the potential of quantitative immunofluorescence for mapping neuroprotective pathways and highlights HSP70’s impact on mitigating FUS-related pathology in ALS.
Publication DOI: | https://doi.org/10.3390/app142411614 |
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Divisions: | College of Engineering & Physical Sciences > School of Engineering and Technology > Mechanical, Biomedical & Design College of Engineering & Physical Sciences > Engineering for Health College of Engineering & Physical Sciences |
Funding Information: | This research was financed by a grant from the Russian Science Foundation No. 23-25-00448 from 12.01.2023 (https://rscf.ru/project/23-25-00448/) |
Additional Information: | Copyright © 2024 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
Uncontrolled Keywords: | heat shock proteins,HSP70,HSPA1A,neurodegeneration,primary motor cortex,amyotrophic lateral sclerosis,ALS,fused in sarcoma,FUS,transgenic animals,immunofluorescence,microscopy |
Publication ISSN: | 2076-3417 |
Last Modified: | 31 Mar 2025 07:27 |
Date Deposited: | 03 Jan 2025 16:16 |
Full Text Link: | |
Related URLs: |
https://www.mdp ... 417/14/24/11614
(Publisher URL) http://www.scop ... tnerID=8YFLogxK (Scopus URL) |
PURE Output Type: | Article |
Published Date: | 2024-12 |
Published Online Date: | 2024-12-12 |
Accepted Date: | 2024-12-11 |
Authors: |
Piavchenko, Gennadii A.
Pokidova, Ksenia S. Kuzmin, Egor A. Venediktov, Artem A. Izmailov, Ilya Y. Meglinski, Igor V. ( ![]() Kuznetsov, Sergey L. |