journal article·2024-12-12Open access

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

Gennadii A. Piavchenko, Ksenia S. Pokidova, Egor A. Kuzmin, Artem A. Venediktov, Ilya Y. Izmailov, Igor V. Meglinski, Sergey L. Kuznetsov

Applied Sciences· Volume 14· Issue 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.
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Research context

OpenAlex topics: Heat shock proteins research · Endoplasmic Reticulum Stress and Disease · Genetics, Aging, and Longevity in Model Organisms

SciVX discovery tags are terminology-based. OpenAlex topics are source-provided classifications.

Affiliations

Department of Human Anatomy and Histology, I. M. Sechenov First Moscow State Medical University (Sechenov University), 11/10, Mokhovaya Street, 125009 Moscow, Russia

College of Engineering and Physical Sciences, Aston University, Birmingham B4 7ET, UK

Funding

Russian Science Foundation

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