Peker Nesibe, Sharma Mridula, Kambadur Ravi
Koç University Research Center for Translational Medicine (KUTTAM), Koç University, Istanbul, 34010, Turkey.
School of Biological Sciences, Nanyang Technological University, 60 Nanyang Drive, 637551, Singapore.
NPJ Parkinsons Dis. 2022 Nov 17;8(1):159. doi: 10.1038/s41531-022-00419-3.
Parkinson's Disease (PD) is a chronic and progressive neurodegenerative disease manifesting itself with tremors, muscle stiffness, bradykinesia, dementia, and depression. Mutations of mitochondrial E3 ligase, PARKIN, have been associated with juvenile PD. Previous studies have characterized muscle atrophy and motor deficits upon loss of functional Parkin in fly and rodent models. However, the mechanisms behind pathophysiology of Parkin deficient muscle remains to be elusive. Here, results suggested that knock down of Parkin significantly increases proteolytic activities in skeletal muscle cell line, the C2C12 myotubes. However, the atrogene levels increase moderately in Parkin deficient cell line. To further investigate the role of Parkin in skeletal muscle atrophy, Parkin knock out (KO) and wild type mice were subjected to 48 h starvation. After 48 h fasting, a greater reduction in skeletal muscle weights was observed in Parkin KO mice as compared to age matched wild type control, suggesting elevated proteolytic activity in the absence of Parkin. Subsequent microarray analyses revealed further enhanced expression of FOXO and ubiquitin pathway in fasted Parkin KO mice. Furthermore, a greater reduction in the expression of cytoskeleton genes was observed in Parkin KO mice following 48 h fasting. Collectively, these results suggest that Parkin deficiency exacerbates fasting-induced skeletal muscle wasting, through upregulating genes involved in catabolic activities in skeletal muscle.
帕金森病(PD)是一种慢性进行性神经退行性疾病,表现为震颤、肌肉僵硬、运动迟缓、痴呆和抑郁。线粒体E3连接酶PARKIN的突变与青少年帕金森病有关。先前的研究已经在果蝇和啮齿动物模型中描述了功能性Parkin缺失后的肌肉萎缩和运动缺陷。然而,Parkin缺陷型肌肉病理生理学背后的机制仍然难以捉摸。在这里,结果表明,敲低Parkin会显著增加骨骼肌细胞系C2C12肌管中的蛋白水解活性。然而,在Parkin缺陷型细胞系中,萎缩基因水平适度增加。为了进一步研究Parkin在骨骼肌萎缩中的作用,对Parkin基因敲除(KO)小鼠和野生型小鼠进行了48小时的饥饿处理。禁食48小时后,与年龄匹配的野生型对照相比,Parkin KO小鼠的骨骼肌重量下降幅度更大,这表明在没有Parkin的情况下蛋白水解活性升高。随后的微阵列分析显示,禁食的Parkin KO小鼠中FOXO和泛素途径的表达进一步增强。此外,禁食48小时后,Parkin KO小鼠中细胞骨架基因的表达下降幅度更大。总的来说,这些结果表明,Parkin缺乏通过上调参与骨骼肌分解代谢活动的基因,加剧了禁食诱导的骨骼肌萎缩。