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dc.contributor.authorKwak, Dongminen_US
dc.contributor.authorWei, Guoxianen_US
dc.contributor.authorThompson, LaDora V.en_US
dc.contributor.authorKim, Jong-Heeen_US
dc.coverage.spatialSwitzerlanden_US
dc.date2020-07-17
dc.date.accessioned2021-02-22T18:30:01Z
dc.date.available2021-02-22T18:30:01Z
dc.date.issued2020-07-19
dc.identifierhttps://www.ncbi.nlm.nih.gov/pubmed/32707682
dc.identifier.citationDongmin 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
dc.identifier.issn1660-4601
dc.identifier.urihttps://hdl.handle.net/2144/42077
dc.description.abstractDuchenne 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.en_US
dc.languageeng
dc.language.isoen_US
dc.relation.ispartofInt J Environ Res Public Health
dc.rightsCopyright © 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/).en_US
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.subject7-week old Mdx miceen_US
dc.subjectDuchenne muscular dystrophyen_US
dc.subjectLMP7en_US
dc.subjectONX-0914en_US
dc.subjectImmunoproteasomeen_US
dc.subjectInflammationen_US
dc.subjectMotor performanceen_US
dc.subjectAnimalsen_US
dc.subjectDisease models, animalen_US
dc.subjectDystrophinen_US
dc.subjectHumansen_US
dc.subjectMiceen_US
dc.subjectMice, inbred C57BLen_US
dc.subjectMice, inbred mdxen_US
dc.subjectMuscle, skeletalen_US
dc.subjectOligopeptidesen_US
dc.subjectPhenotypeen_US
dc.subjectYoung adulten_US
dc.subjectToxicologyen_US
dc.titleShort-term ONX-0914 administration: performance and muscle phenotype in Mdx miceen_US
dc.typeArticleen_US
dc.description.versionPublished versionen_US
dc.identifier.doi10.3390/ijerph17145211
pubs.elements-sourcepubmeden_US
pubs.notesEmbargo: Not knownen_US
pubs.organisational-groupBoston Universityen_US
pubs.organisational-groupBoston University, College of Health & Rehabilitation Sciences: Sargent Collegeen_US
pubs.organisational-groupBoston University, College of Health & Rehabilitation Sciences: Sargent College, Physical Therapy and Athletic Trainingen_US
pubs.publication-statusPublished onlineen_US
dc.identifier.orcid0000-0001-5115-3697 (Wei, Guoxian)
dc.identifier.mycv564690


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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/).
Except where otherwise noted, this item's license is described as 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/).