颗粒蛋白前体基因传递可保护帕金森病小鼠模型中的多巴胺能神经元。
Progranulin gene delivery protects dopaminergic neurons in a mouse model of Parkinson's disease.
作者信息
Van Kampen Jackalina M, Baranowski David, Kay Denis G
机构信息
Neurodyn Inc., Charlottetown, Prince Edward Island, Canada; Department of Biomedical Science, University of Prince Edward Island, Charlottetown, Prince Edward Island, Canada.
Neurodyn Inc., Charlottetown, Prince Edward Island, Canada.
出版信息
PLoS One. 2014 May 7;9(5):e97032. doi: 10.1371/journal.pone.0097032. eCollection 2014.
Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by tremor, rigidity and akinesia/bradykinesia resulting from the progressive loss of nigrostriatal dopaminergic neurons. To date, only symptomatic treatment is available for PD patients, with no effective means of slowing or stopping the progression of the disease. Progranulin (PGRN) is a 593 amino acid multifunction protein that is widely distributed throughout the CNS, localized primarily in neurons and microglia. PGRN has been demonstrated to be a potent regulator of neuroinflammation and also acts as an autocrine neurotrophic factor, important for long-term neuronal survival. Thus, enhancing PGRN expression may strengthen the cells resistance to disease. In the present study, we have used the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) model of PD to investigate the possible use of PGRN gene delivery as a therapy for the prevention or treatment of PD. Viral vector delivery of the PGRN gene was an effective means of elevating PGRN expression in nigrostriatal neurons. When PGRN expression was elevated in the SNC, nigrostriatal neurons were protected from MPTP toxicity in mice, along with a preservation of striatal dopamine content and turnover. Further, protection of nigrostriatal neurons by PGRN gene therapy was accompanied by reductions in markers of MPTP-induced inflammation and apoptosis as well as a complete preservation of locomotor function. We conclude that PGRN gene therapy may have beneficial effects in the treatment of PD.
帕金森病(PD)是一种进行性神经退行性疾病,其特征为震颤、僵硬以及运动不能/运动迟缓,这些症状是由黑质纹状体多巴胺能神经元的逐渐丧失所致。迄今为止,PD患者仅能接受对症治疗,尚无有效方法减缓或阻止疾病进展。前颗粒蛋白(PGRN)是一种由593个氨基酸组成的多功能蛋白,广泛分布于中枢神经系统,主要定位于神经元和小胶质细胞。PGRN已被证明是神经炎症的有效调节因子,并且作为一种自分泌神经营养因子,对神经元的长期存活至关重要。因此,增强PGRN表达可能会增强细胞对疾病的抵抗力。在本研究中,我们使用了PD的1-甲基-4-苯基-1,2,3,6-四氢吡啶(MPTP)模型,以研究PGRN基因递送作为预防或治疗PD的一种疗法的可能性。通过病毒载体递送PGRN基因是提高黑质纹状体神经元中PGRN表达的有效手段。当在黑质致密部(SNC)中PGRN表达升高时,小鼠黑质纹状体神经元免受MPTP毒性影响,同时纹状体多巴胺含量和周转率得以保留。此外,PGRN基因疗法对黑质纹状体神经元的保护作用伴随着MPTP诱导的炎症和凋亡标志物的减少以及运动功能的完全保留。我们得出结论,PGRN基因疗法可能对PD治疗具有有益效果。
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