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Abstract：
Connective tissue growth factor or cellular communication network 2 (CCN2/CTGF) is a matricellular protein member of the CCN family involved in several crucial biological processes. In skeletal muscle, CCN2/CTGF abundance is elevated in human muscle biopsies and/or animal models for diverse neuromuscular pathologies, including muscular dystrophies, neurodegenerative disorders, muscle denervation, and muscle overuse. In this context, CCN2/CTGF is deeply involved in extracellular matrix (ECM) modulation, acting as a strong pro-fibrotic factor that promotes excessive ECM accumulation. Reducing CCN2/CTGF levels or biological activity in pathological conditions can decrease fibrosis, improve muscle architecture and function. In this work, we summarize information about the role of CCN2/CTGF in fibrosis associated with neuromuscular pathologies and the mechanisms and signaling pathways that regulate their expression in skeletal muscle.
摘要：
结缔组织生长因子或细胞通讯网络2（CCN2/CTGF）是CCN家族的基质细胞蛋白成员，参与几个关键的生物学过程。在骨骼肌中，CCN2/CTGF丰度在人类肌肉活检和/或不同神经肌肉病理的动物模型中升高。包括肌营养不良,神经退行性疾病，肌肉神经支配,肌肉过度使用。在这种情况下，CCN2/CTGF深入参与细胞外基质(ECM)的调制,作为促进ECM过度积累的强促纤维化因子。在病理条件下降低CCN2/CTGF水平或生物活性可以减少纤维化，改善肌肉结构和功能。在这项工作中,我们总结了CCN2/CTGF在与神经肌肉病变相关的纤维化中的作用以及调节其在骨骼肌中表达的机制和信号通路。
