Quantitative Immunofluorescence Mapping of HSP70’s Neuroprotective Effects in FUS-ALS Mouse Models

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
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. (ORCID Profile 0000-0002-7613-8191)
Kuznetsov, Sergey L.

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