Stem cell therapy and FUS[1‐359]‐transgenic mice: A recent study highlighting a promising ALS model and a promising therapy

Retinal damage is an optional sign in a number of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). The aim of this work was to assess the structural and functional state of the retina in a murine model of ALS caused by overexpression of the aberrant FUS protein [1-359]. The retinal examination was carried out on 12 transgenic and 13 wild-type mice of 2.5–3 months of age. The study revealed not statistically significant higher level of ophthalmoscopic violations in FUS[1-359] mice. Moreover, gene expression assay confirmed an increased expression of the inflammatory genes Vegfa, Il1b, Il6, Icam1, Tnfa. However, despite the detected structural and functional abnormalities, western blot analysis and quantitative PCR did not detect the expression of the protein and mRNA products of the FUS transgene in the retina of FUS[1-359] mice.

show abstract

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

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.

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

customersupport@researchsolutions.com

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Stem cell therapy and FUS[1‐359]‐transgenic mice: A recent study highlighting a promising ALS model and a promising therapy

Cited by 3 publications

References 10 publications

In FUS[1−359]‐tg mice O,S-dibenzoyl thiamine reduces muscle atrophy, decreases glycogen synthase kinase 3 beta, and normalizes the metabolome

In FUS[1−359]‐tg mice O,S-dibenzoyl thiamine reduces muscle atrophy, decreases glycogen synthase kinase 3 beta, and normalizes the metabolome

Retinal abnormalities in transgenic mice overexpressing aberrant human FUS[1-359] gene

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

Contact Info

Product

Resources

About