Immunoproteasome Inhibition Ameliorates Aged Dystrophic Mouse Muscle Environment.

ABSTRACT: Muscle wasting is a major pathological feature observed in Duchenne muscular dystrophy (DMD) and is the result of the concerted effects of inflammation, oxidative stress and cell senescence. The inducible form of proteasome, or immunoproteasome (IP), is involved in all the above mentioned processes, regulating antigen presentation, cytokine production and immune cell response. IP inhibition has been previously shown to dampen the altered molecular, histological and functional features of 3-month-old mdx mice, the animal model for DMD. In this study, we described the role of ONX-0914, a selective inhibitor of the PSMB8 subunit of immunoproteasome, in ameliorating the pathological traits that could promote muscle wasting progression in older, 9-month-old mdx mice. ONX-0914 reduces the number

SUBMITTER: Tripodi L 

PROVIDER: S-EPMC9739773 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature