G-CSF supports long-term muscle regeneration in mouse models of muscular dystrophy
G-CSF supports long-term muscle regeneration in mouse models of muscular dystrophy
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DOI：
10.1038/ncomms7745
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发表时间：
2015-04-01
影响因子：
16.6
通讯作者：
Fukuda, Keiichi
中科院分区：
文献类型：
--
作者：
Hayashiji, Nozomi;Yuasa, Shinsuke;Fukuda, Keiichi
杜氏肌营养不良症（DMD）是一种慢性和危及生命的疾病，最初由肌肉再生支持，但最终显示卫星细胞衰竭和肌肉功能障碍。骨骼肌稳态的终身维持需要卫星干细胞库的保存。不对称细胞分裂在卫星细胞库的维持中起着关键作用。在这里，我们表明，粒细胞集落刺激因子受体（G-CSFR）是不对称表达的活化卫星细胞。G-CSF在离体培养的纤维中的多个分化阶段期间对卫星细胞群体产生积极影响。G-CSF可能是重要的，在开发一种有效的治疗DMD的基础上，其潜力，以调节多个阶段的再生肌细胞的供应。这项研究表明，G-CSF-G-CSFR轴对于不同严重程度的DMD小鼠模型的长期肌肉再生，功能维持和寿命延长至关重要。
Duchenne muscular dystrophy (DMD) is a chronic and life-threatening disease that is initially supported by muscle regeneration but eventually shows satellite cell exhaustion and muscular dysfunction. The life-long maintenance of skeletal muscle homoeostasis requires the satellite stem cell pool to be preserved. Asymmetric cell division plays a pivotal role in the maintenance of the satellite cell pool. Here we show that granulocyte colony-stimulating factor receptor (G-CSFR) is asymmetrically expressed in activated satellite cells. G-CSF positively affects the satellite cell population during multiple stages of differentiation in ex vivo cultured fibres. G-CSF could be important in developing an effective therapy for DMD based on its potential to modulate the supply of multiple stages of regenerated myocytes. This study shows that the G-CSF-G-CSFR axis is fundamentally important for long-term muscle regeneration, functional maintenance and lifespan extension in mouse models of DMD with varying severities.