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首页> 美国卫生研究院文献>International Journal of Molecular Sciences >Abnormal NFAT5 Physiology in Duchenne Muscular Dystrophy Fibroblasts as a Putative Explanation for the Permanent Fibrosis Formation in Duchenne Muscular Dystrophy
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Abnormal NFAT5 Physiology in Duchenne Muscular Dystrophy Fibroblasts as a Putative Explanation for the Permanent Fibrosis Formation in Duchenne Muscular Dystrophy

机译：Duchenne肌营养不良成纤维细胞的异常NFAT5生理学作为Duchenne肌营养不良的永久纤维化形成的推定解释

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摘要

Duchenne muscular dystrophy (DMD) is characterized by chronic inflammation and fibrotic tissue production by fibroblasts. The promyogenic factor nuclear factor of activated T-cells 5 (NFAT5) is virtually present in all cells, responding to hyperosmolar or pro-inflammatory stress. In embryogenic fibroblasts, absence of NFAT5 results in cell cycle arrest. Here, unaffected skeletal muscle fibroblasts from one healthy donor showed NFAT5 nuclear translocation upon hyperosmolar stress and normal cell viability. Absence of NFAT5 translocation under pro-inflammatory conditions resulted in decreased cell growth (Incucyte ZOOM). In DMD skeletal muscle fibroblasts from one DMD patient, NFAT5 was merely located in the nucleus. Exposure to hyperosmolar conditions or pro-inflammatory cytokines IFN-γ, IL-1β and TNF-α had no influence on NFAT5 physiology (immunofluorescence, western blotting, RT-qPCR). Hyperosmolarity resulted in decreased cell viability and pro-inflammatory stress in unaltered cell growth. These findings suggest that NFAT5 is vital to DMD fibroblast survival. Exposure to pro-inflammatory or hyperosmolar stress in DMD fibroblasts results in an unexpected NFAT5 response, where fibroblasts are not triggered by inflammatory cytokines and do not withstand hyperosmolarity. Chronic inflammation could be viewed as a non-restrictive factor in the formation of fibrosis in DMD. Abnormal NFAT5 physiology could provide a molecular explanation for permanent fibrotic matrix production by DMD fibroblasts.
机译：Duchenne肌营养不良（DMD）的特征在于成纤维细胞慢性炎症和纤维化组织产生。活化T细胞5（NFAT5）的初始因子核因子实际上存在于所有细胞中，响应Hyperosmolar或促炎胁迫。在胚胎成纤维细胞中，没有NFAT5导致细胞周期停滞。这里，来自一个健康供体的未受影响的骨骼肌成纤维细胞显示出高摩托胁迫和正常细胞活力的NFAT5核易位。在促炎症条件下没有NFAT5易位导致细胞生长减少（酸性变焦）。在来自一个DMD患者的DMD骨骼肌成纤维细胞中，NFAT5仅位于细胞核中。暴露于高氧化镁条件或促炎细胞因子IFN-γ，IL-1β和TNF-α对NFAT5生理学（免疫荧光，蛋白质印迹，RT-QPCR）没有影响。高摩洛度导致细胞活力下降和促炎细胞生长中的促炎胁迫。这些发现表明NFAT5对DMD成纤维细胞存活至关重要。暴露于DMD成纤维细胞中的促炎或高氧化铝应激导致意外的NFAT5反应，其中成纤维细胞不会被炎性细胞因子触发，并且不抵抗过氧性。慢性炎症可以被视为在DMD中纤维化形成的非限制性因素。 NFAT5生理学异常可以提供DMD成纤维细胞的永久性纤维化基质产生的分子解释。

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