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Animal Models of FUS-Proteinopathy: A Systematic Review

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Abstract

Mutations that disrupt the function of the DNA/RNA-binding protein FUS could cause amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. One of the key features in ALS pathogenesis is the formation of insoluble protein aggregates containing aberrant isoforms of the FUS protein in the cytoplasm of upper and lower motor neurons. Reproduction of human pathology in animal models is the main tool for studying FUS-associated pathology and searching for potential therapeutic agents for ALS treatment. In this review, we provide a systematic analysis of the role of FUS protein in ALS pathogenesis and an overview of the results of modelling FUS-proteinopathy in animals.

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Funding

The study was financially supported by the Russian Science Foundation (project no. 22-74-10050).

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Authors and Affiliations

  1. Engelhardt Institute of Molecular Biology, Russian Academy of Sciences, 119991, Moscow, Russia

    Alexander Rezvykh & Sergei Funikov

  2. Institute of Physiologically Active Compounds, Federal Research Center of Problems of Chemical Physics and Medicinal Chemistry, Russian Academy of Sciences, 142432, Chernogolovka, Russia

    Daniil Shteinberg & Aleksey Ustyugov

  3. State University of Education, 141014, Mytishchi, Russia

    Evgeny Bronovitsky

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  1. Alexander Rezvykh
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  2. Daniil Shteinberg
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Contributions

A.R. and D.Sh. wrote the manuscript. E.B. prepared the section “Prospects for the Study of FUS-Proteinopathy and Treatment of ALS”. A.U. and S.F. proposed a general manuscript concept and edited the manuscript.

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Correspondence to Sergei Funikov.

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Translated from Uspekhi Biologicheskoi Khimii, 2024, Vol. 64, pp. 73-116.

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Rezvykh, A., Shteinberg, D., Bronovitsky, E. et al. Animal Models of FUS-Proteinopathy: A Systematic Review. Biochemistry Moscow 89 (Suppl 1), S34–S56 (2024). https://doi.org/10.1134/S0006297924140037

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