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

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

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.

HEAT shock proteins; STEREOLOGY; MOTOR cortex; TRANSGENIC mice; MOLECULAR chaperones; AMYOTROPHIC lateral sclerosis

Applied Sciences (2076-3417), 2024, Vol 14, Issue 24, p11614

2076-3417

Academic Journal

10.3390/app142411614