Clinical Biochemistry and Clinical Molecular Biology Unit, Department of Experimental and Clinical Biomedical Sciences "Mario Serio", University of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
Interuniversity Institute of Myology, University of Firenze, 50134 Firenze, Italy.
Int J Mol Sci. 2019 Dec 17;20(24):6364. doi: 10.3390/ijms20246364.
The bioactive sphingolipid metabolite, sphingosine 1-phosphate (S1P), and the signaling pathways triggered by its binding to specific G protein-coupled receptors play a critical regulatory role in many pathophysiological processes, including skeletal muscle and nervous system degeneration. The signaling transduced by S1P binding appears to be much more complex than previously thought, with important implications for clinical applications and for personalized medicine. In particular, the understanding of S1P/S1P receptor signaling functions in specific compartmentalized locations of the cell is worthy of being better investigated, because in various circumstances it might be crucial for the development or/and the progression of neuromuscular diseases, such as Charcot-Marie-Tooth disease, myasthenia gravis, and Duchenne muscular dystrophy.
生物活性神经鞘脂代谢物，即 1-磷酸鞘氨醇（S1P），以及其与特定 G 蛋白偶联受体结合所引发的信号通路，在多种生理病理过程中发挥关键调节作用，包括骨骼肌和神经系统退化。S1P 结合所传递的信号似乎比之前认为的要复杂得多，这对临床应用和个性化医疗具有重要意义。特别是，S1P/S1P 受体信号在细胞特定分隔区域的功能的理解值得更深入研究，因为在各种情况下，它可能对神经肌肉疾病（如 Charcot-Marie-Tooth 病、重症肌无力和杜氏肌营养不良症）的发展和/或进展至关重要。
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