Department of Cell Biology and Anatomical Sciences, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Department of Stem Cells and Regenerative Medicine, Faculty of Medical Biotechnology, National Institute of Genetic Engineering and Biotechnology, Tehran, Iran.
J Mol Neurosci. 2018 May;65(1):17-27. doi: 10.1007/s12031-018-1062-x. Epub 2018 Apr 21.
Huntington's disease (HD) is a genetically heritable disorder, linked with continuing cell loss and degeneration mostly in the striatum. Currently, cell therapy approaches in HD have essentially been focused on replenishing or shielding cells lost over the period of the disease. Herein, we sought to explore the in vitro and in vivo efficacy of primary rat Sertoli cells (SCs) and their paracrine effect against oxidative stress with emphasis on HD. Initially, SCs were isolated and immunophenotypically characterized by positive expression of GATA4. Besides, synthesis of neurotrophic factors of glial cell-derived neurotrophic factor and VEGF by SCs were proved. Next, PC12 cells were exposed to hydrogen peroxide in the presence of conditioned media (CM) collected from SC (SC-CM) and cell viability and neuritogenesis were determined. Bilateral striatal implantation of SC in 3-nitropropionic acid (3-NP)-lesioned rat models was performed, and 1 month later, post-graft analysis was done. According to our in vitro results, the CM of SC protected PC12 cells against oxidative stress and remarkably augmented cell viability and neurite outgrowth. Moreover, grafted SCs survived, exhibited decreases in both gliosis and inflammatory cytokine levels, and ameliorated motor coordination and muscle activity, together with an increase in striatal volume as well as in dendritic length of the striatum in HD rats. In conclusion, our results indicate that SCs provide a supportive environment, with potential therapeutic benefits aimed at HD.
亨廷顿病(HD)是一种遗传性疾病,主要与纹状体中持续的细胞丢失和退化有关。目前,HD 中的细胞治疗方法主要集中在补充或保护疾病期间丢失的细胞。在此,我们试图探索原代大鼠支持细胞(SCs)的体外和体内功效及其对氧化应激的旁分泌作用,重点是 HD。最初,通过 GATA4 的阳性表达来分离和免疫表型鉴定 SC,并证明了 SC 合成神经营养因子胶质细胞源性神经营养因子和 VEGF。接下来,将 PC12 细胞暴露于过氧化氢中,并使用从 SC(SC-CM)收集的条件培养基(CM),然后测定细胞活力和神经突形成。在 3-硝基丙酸(3-NP)损伤的大鼠模型中进行双侧纹状体植入 SC,1 个月后进行移植后分析。根据我们的体外结果,SC 的 CM 可保护 PC12 细胞免受氧化应激,并显著提高细胞活力和神经突生长。此外,移植的 SC 存活,减少了神经胶质增生和炎症细胞因子水平,并改善了运动协调和肌肉活动,同时增加了 HD 大鼠纹状体的体积和纹状体的树突长度。总之,我们的结果表明,SC 提供了一个支持性环境,具有针对 HD 的潜在治疗益处。