Neuroregeneration and Stem Cell Programs, Institute for Cell Engineering, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Proc Natl Acad Sci U S A. 2022 Sep 6;119(36):e2204835119. doi: 10.1073/pnas.2204835119. Epub 2022 Aug 31.
Physical activity provides clinical benefit in Parkinson's disease (PD). Irisin is an exercise-induced polypeptide secreted by skeletal muscle that crosses the blood-brain barrier and mediates certain effects of exercise. Here, we show that irisin prevents pathologic α-synuclein (α-syn)-induced neurodegeneration in the α-syn preformed fibril (PFF) mouse model of sporadic PD. Intravenous delivery of irisin via viral vectors following the stereotaxic intrastriatal injection of α-syn PFF cause a reduction in the formation of pathologic α-syn and prevented the loss of dopamine neurons and lowering of striatal dopamine. Irisin also substantially reduced the α-syn PFF-induced motor deficits as assessed behaviorally by the pole and grip strength test. Recombinant sustained irisin treatment of primary cortical neurons attenuated α-syn PFF toxicity by reducing the formation of phosphorylated serine 129 of α-syn and neuronal cell death. Tandem mass spectrometry and biochemical analysis revealed that irisin reduced pathologic α-syn by enhancing endolysosomal degradation of pathologic α-syn. Our findings highlight the potential for therapeutic disease modification of irisin in PD.
体育活动对帕金森病(PD)有临床益处。鸢尾素是一种由骨骼肌分泌的运动诱导多肽,可穿过血脑屏障并介导运动的某些作用。在这里,我们表明鸢尾素可预防散发性 PD 的α-突触核蛋白(α-syn)预形成纤维(PFF)小鼠模型中的病理性α-syn 诱导的神经退行性变。在立体定向纹状体内注射α-syn PFF 后,通过病毒载体静脉内递送达尔文可减少病理性α-syn 的形成,并防止多巴胺神经元丢失和纹状体多巴胺降低。鸢尾素还通过杆和握力测试在行为上大大减少了α-syn PFF 诱导的运动缺陷。重组持续的鸢尾素处理原代皮质神经元可通过减少磷酸化丝氨酸 129 的α-syn 的形成和神经元细胞死亡来减轻α-syn PFF 毒性。串联质谱和生化分析表明,鸢尾素通过增强病理性α-syn 的内溶酶体降解来减少病理性α-syn。我们的发现强调了在 PD 中使用鸢尾素进行治疗性疾病修饰的潜力。