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The accumulation of muscle RING finger-1 in regenerating myofibers: Implications for muscle repair in immune-mediated necrotizing myopathy

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单位: [1]Huazhong Univ Sci & Technol,Tongji Hosp,Dept Neurol,Tongji Med Coll,Wuhan,Peoples R China [2]Huazhong Univ Sci & Technol,Tongji Hosp,Genet Diagnost Ctr,Dept Internal Med,Tongji Med Coll,Wuhan,Peoples R China
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关键词: muscle RING finger-1 immune-mediated necrotizing myopathy dermatomyositis dysferlinopathy regeneration

摘要:
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.

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出版当年[2021]版:
大类 | 3 区 医学
小类 | 3 区 临床神经病学 3 区 神经科学
最新[2025]版:
大类 | 3 区 医学
小类 | 3 区 临床神经病学 3 区 神经科学
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出版当年[2020]版:
Q2 NEUROSCIENCES Q2 CLINICAL NEUROLOGY
最新[2023]版:
Q2 CLINICAL NEUROLOGY Q3 NEUROSCIENCES

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第一作者单位: [1]Huazhong Univ Sci & Technol,Tongji Hosp,Dept Neurol,Tongji Med Coll,Wuhan,Peoples R China
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