Lipopolysaccharide triggers exacerbated microglial activation, excessive cytokine release and behavioural disturbances in mice with truncated Fused-in-Sarcoma Protein (FUS)

Trofimov, Alexander; Pavlov, Dmitrii; Goswami, Anand; Gorlova, Anna; Chaprov, Kirill; Umriukhin, Aleksei; Kalueff, Allan; Deykin, Alexey; Lesch, Klaus-Peter; Anthony, Daniel Clive; Strekalova, Tatyana

doi:10.1016/j.bbih.2023.100686

Brain, Behavior, & Immunity - Health

2023

DOI: 10.1016/j.bbih.2023.100686

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Lipopolysaccharide triggers exacerbated microglial activation, excessive cytokine release and behavioural disturbances in mice with truncated Fused-in-Sarcoma Protein (FUS)

Alexander Trofimov,

Dmitrii Pavlov,

Anand Goswami

et al.

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2024

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

Piavchenko,

Pokidova,

Kuzmin

et al. 2024

Applied Sciences

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

Piavchenko,

Pokidova,

Kuzmin

et al. 2024

Applied Sciences

View full text Add to dashboard Cite

show abstract

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Lipopolysaccharide triggers exacerbated microglial activation, excessive cytokine release and behavioural disturbances in mice with truncated Fused-in-Sarcoma Protein (FUS)

Cited by 1 publication

References 38 publications

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

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

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