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牛磺酸在骨骼肌功能中的作用及其作为杜氏肌营养不良症支持性治疗方法的潜力。

The Role of Taurine in Skeletal Muscle Functioning and Its Potential as a Supportive Treatment for Duchenne Muscular Dystrophy.

作者信息

Merckx Caroline, De Paepe Boel

机构信息

Department of Neurology, Ghent University Hospital, 9000 Ghent, Belgium.

Neuromuscular Reference Center, Ghent University Hospital, 9000 Ghent, Belgium.

出版信息

Metabolites. 2022 Feb 19;12(2):193. doi: 10.3390/metabo12020193.

Abstract

Taurine (2-aminoethanesulfonic acid) is required for ensuring proper muscle functioning. Knockout of the taurine transporter in mice results in low taurine concentrations in the muscle and associates with myofiber necrosis and diminished exercise capacity. Interestingly, regulation of taurine and its transporter is altered in the mdx mouse, a model for Duchenne Muscular Dystrophy (DMD). DMD is a genetic disorder characterized by progressive muscle degeneration and weakness due to the absence of dystrophin from the muscle membrane, causing destabilization and contraction-induced muscle cell damage. This review explores the physiological role of taurine in skeletal muscle and the consequences of a disturbed balance in DMD. Its potential as a supportive treatment for DMD is also discussed. In addition to genetic correction, that is currently under development as a curative treatment, taurine supplementation has the potential to reduce muscle inflammation and improve muscle strength in patients.

摘要

牛磺酸(2-氨基乙磺酸)对于确保肌肉正常功能是必需的。敲除小鼠体内的牛磺酸转运体,会导致肌肉中牛磺酸浓度降低,并伴有肌纤维坏死和运动能力下降。有趣的是,在杜氏肌营养不良症(DMD)模型mdx小鼠中,牛磺酸及其转运体的调节发生了改变。DMD是一种遗传性疾病,其特征是由于肌膜中缺乏抗肌萎缩蛋白,导致进行性肌肉退化和无力,进而引起肌肉细胞不稳定和收缩诱导性损伤。本文综述探讨了牛磺酸在骨骼肌中的生理作用以及DMD中平衡失调的后果。还讨论了其作为DMD支持性治疗的潜力。除了目前正在开发作为治愈性治疗的基因校正外,补充牛磺酸有可能减轻患者的肌肉炎症并提高肌肉力量。

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