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    Short-term ONX-0914 administration: performance and muscle phenotype in Mdx mice

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    Copyright © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
    Date Issued
    2020-07-19
    Publisher Version
    10.3390/ijerph17145211
    Author(s)
    Kwak, Dongmin
    Wei, Guoxian
    Thompson, LaDora V.
    Kim, Jong-Hee
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    Permanent Link
    https://hdl.handle.net/2144/42077
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    Published version
    Citation (published version)
    Dongmin Kwak, Guoxian Wei, LaDora V Thompson, Jong-Hee Kim. 2020. "Short-Term ONX-0914 Administration: Performance and Muscle Phenotype in Mdx Mice.." Int J Environ Res Public Health, Volume 17, Issue 14, https://doi.org/10.3390/ijerph17145211
    Abstract
    Duchenne muscular dystrophy (DMD) is a severe muscle-wasting disease. Although the lack of dystrophin protein is the primary defect responsible for the development of DMD, secondary disease complications such as persistent inflammation contribute greatly to the pathogenesis and the time-dependent progression of muscle destruction. The immunoproteasome is a potential therapeutic target for conditions or diseases mechanistically linked to inflammation. In this study, we explored the possible effects of ONX-0914 administration, an inhibitor specific for the immunoproteasome subunit LMP7 (ß5i), on motor performance, muscular pathology and protein degradation in 7-week old MDX mice, an age when the dystrophic muscles show extensive degeneration and regeneration. ONX-0914 (10 mg/kg) was injected subcutaneously on Day 2, 4, and 6. The mice were evaluated for physical performance (walking speed and strength) on Day 1 and 8. We show that this short-term treatment of ONX-0914 in MDX mice did not alter strength nor walking speed. The physical performance findings were consistent with no change in muscle inflammatory infiltration, percentage of central nuclei and proteasome content. Taken together, muscle structure and function in the young adult MDX mouse model are not altered with ONX-0914 treatment, indicating the administration of ONX-0914 during this critical time period does not exhibit any detrimental effects and may be an effective treatment of secondary complications of muscular dystrophy after further investigations.
    Rights
    Copyright © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
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    • SAR: Physical Therapy & Athletic Training: Scholarly Papers [52]
    • BU Open Access Articles [3730]


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