BackgroundMuscle RING finger-1 (MuRF-1) plays a key role in the degradation of skeletal muscle proteins. We hypothesize the involvement of MuRF-1 in immune-mediated necrotizing myopathy (IMNM). MethodsMuscle biopsies from patients with IMNM (n = 37) were analyzed and compared to biopsies from patients with dermatomyositis (DM, n = 13), dysferlinopathy (n = 9) and controls (n = 7) using immunostaining. ResultsMuRF-1 staining could be observed in IMNM, DM and dysferlinopathy biopsies, whereas the percentage of MuRF-1 positive myofibers was significantly higher in IMNM than in dysferlinopathy (p = 0.0448), and positively correlated with muscle weakness and disease activity in IMNM and DM. Surprisingly, MuRF-1 staining predominantly presented in regenerating fibers but not in atrophic fibers. Moreover, MuRF-1-positive fibers tended to be distributed around necrotic myofibers and myofibers with sarcolemma membrane attack complex deposition. Abundant MuRF-1 expression in IMNM and DM was associated with rapid activation of myogenesis after muscle injury, whereas relatively low expression of MuRF-1 in dysferlinopathy may be attributed to damaged muscle regeneration. ConclusionsMuRF-1 accumulated in regenerating myofibers, which may contribute to muscle injury repair in IMNM and DM. MuRF-1 staining may help clinicians differentiate IMNM and dysferlinopathy.
基金:
National Natural Science Foundation of China; [81873758]
第一作者单位:[1]Huazhong Univ Sci & Technol,Tongji Hosp,Dept Neurol,Tongji Med Coll,Wuhan,Peoples R China
通讯作者:
推荐引用方式(GB/T 7714):
Yang Meng-Ge,Zhang Qing,Wang Hong,et al.The accumulation of muscle RING finger-1 in regenerating myofibers: Implications for muscle repair in immune-mediated necrotizing myopathy[J].FRONTIERS IN NEUROLOGY.2022,13:doi:10.3389/fneur.2022.1032738.
APA:
Yang, Meng-Ge,Zhang, Qing,Wang, Hong,Ma, Xue,Ji, Suqiong...&Bu, Bitao.(2022).The accumulation of muscle RING finger-1 in regenerating myofibers: Implications for muscle repair in immune-mediated necrotizing myopathy.FRONTIERS IN NEUROLOGY,13,
MLA:
Yang, Meng-Ge,et al."The accumulation of muscle RING finger-1 in regenerating myofibers: Implications for muscle repair in immune-mediated necrotizing myopathy".FRONTIERS IN NEUROLOGY 13.(2022)