Short-Term ONX-0914 Administration: Performance and Muscle Phenotype inMdxMiceopen access
- Authors
- Kwak, Dongmin; Wei, Guoxian; Thompson, LaDora, V; Kim, Jong-Hee
- Issue Date
- Jul-2020
- Publisher
- MDPI
- Keywords
- Duchenne muscular dystrophy; immunoproteasome; LMP7; ONX-0914; motor performance; inflammation; 7-week oldMdxmice
- Citation
- INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH, v.17, no.14, pp.1 - 13
- Indexed
- SCIE
SSCI
SCOPUS
- Journal Title
- INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH
- Volume
- 17
- Number
- 14
- Start Page
- 1
- End Page
- 13
- URI
- https://scholarworks.bwise.kr/hanyang/handle/2021.sw.hanyang/136082
- DOI
- 10.3390/ijerph17145211
- ISSN
- 1661-7827
- 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 oldMDXmice, 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 inMDXmice 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 adultMDXmouse 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.
- Files in This Item
-
- Appears in
Collections - 서울 예술·체육대학 > 서울 체육학과 > 1. Journal Articles
![qrcode](https://api.qrserver.com/v1/create-qr-code/?size=55x55&data=https://scholarworks.bwise.kr/hanyang/handle/2021.sw.hanyang/136082)
Items in ScholarWorks are protected by copyright, with all rights reserved, unless otherwise indicated.