利用中脑发育基因调控多巴胺能神经元维持的帕金森病基因疗法。

IF 5.6 2区 生物学
Jintae Kim, Mi-Yoon Chang
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引用次数: 0

摘要

帕金森病(PD)是第二大最常见的神经退行性疾病。其特征是黑质中多巴胺能(DAnergic)神经元的逐渐丧失和多巴胺(DA)水平的降低,从而导致运动和非运动症状。传统的帕金森病治疗方法旨在缓解症状,但不能延缓疾病的进展。帕金森病基因疗法为改善目前的治疗方法提供了一种很有前景的方法,它有可能减轻明显的帕金森病症状,并比传统疗法产生更少的不良反应。DA替代方法和DA酶表达并不能延缓疾病的进展。然而,DA替代基因疗法,如腺体相关病毒(AAV)-谷氨酸脱羧酶(GAD)和L-氨基酸脱羧酶(AADC)基因疗法,可增加DA递质水平,在早期临床试验中已被证明是安全有效的。旨在减缓疾病进展的疾病改变策略似乎很有效。其中包括针对下游通路、神经营养因子和中脑DA能神经元因子的疗法,所有这些疗法在临床前和临床试验中都显示出潜力。这些方法的重点是维持 DA 能神经元的完整性,而不仅仅是针对 DA 递质水平本身。特别是,Nurr1 和 Foxa2 等关键的中脑发育和维持因子可与邻近胶质细胞协同作用,以旁分泌模式保护 DAnergic 神经元免受各种毒性因子的伤害。用其他候选基因疗法同时靶向 DAnergic 神经元和神经胶质细胞也能取得类似的效果,但还需要深入研究。神经营养因子,如神经营养素、胶质细胞系源性神经营养因子(GDNF)、脑源性神经营养因子(BDNF)和血管内皮生长因子(VEGF),也在研究它们支持 DAnergic 神经元存活的潜力。此外,针对自噬-溶酶体通路、线粒体功能和内质网(ER)应激等关键下游通路的基因疗法也是很有前景的途径。基因编辑和传递技术不断发展,为开发治疗帕金森病的有效基因疗法提供了新的机遇。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Gene Therapy for Parkinson's Disease Using Midbrain Developmental Genes to Regulate Dopaminergic Neuronal Maintenance.

Parkinson's disease (PD) is the second most prevalent neurodegenerative disorder. It is characterized by the progressive loss of dopaminergic (DAnergic) neurons in the substantia nigra and decreased dopamine (DA) levels, which lead to both motor and non-motor symptoms. Conventional PD treatments aim to alleviate symptoms, but do not delay disease progression. PD gene therapy offers a promising approach to improving current treatments, with the potential to alleviate significant PD symptoms and cause fewer adverse effects than conventional therapies. DA replacement approaches and DA enzyme expression do not slow disease progression. However, DA replacement gene therapies, such as adeno-associated virus (AAV)-glutamic acid decarboxylase (GAD) and L-amino acid decarboxylase (AADC) gene therapies, which increase DA transmitter levels, have been demonstrated to be safe and efficient in early-phase clinical trials. Disease-modifying strategies, which aim to slow disease progression, appear to be potent. These include therapies targeting downstream pathways, neurotrophic factors, and midbrain DAnergic neuronal factors, all of which have shown potential in preclinical and clinical trials. These approaches focus on maintaining the integrity of DAnergic neurons, not just targeting the DA transmitter level itself. In particular, critical midbrain developmental and maintenance factors, such as Nurr1 and Foxa2, can interact synergistically with neighboring glia, in a paracrine mode of action, to protect DAnergic neurons against various toxic factors. Similar outcomes could be achieved by targeting both DAnergic neurons and glial cells with other candidate gene therapies, but in-depth research is needed. Neurotrophic factors, such as neurturin, the glial-cell-line-derived neurotrophic factor (GDNF), the brain-derived neurotrophic factor (BDNF), and the vascular endothelial growth factor (VEGF), are also being investigated for their potential to support DAnergic neuron survival. Additionally, gene therapies targeting key downstream pathways, such as the autophagy-lysosome pathway, mitochondrial function, and endoplasmic reticulum (ER) stress, offer promising avenues. Gene editing and delivery techniques continue to evolve, presenting new opportunities to develop effective gene therapies for PD.

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来源期刊
自引率
10.70%
发文量
13472
审稿时长
1.7 months
期刊介绍: The International Journal of Molecular Sciences (ISSN 1422-0067) provides an advanced forum for chemistry, molecular physics (chemical physics and physical chemistry) and molecular biology. It publishes research articles, reviews, communications and short notes. Our aim is to encourage scientists to publish their theoretical and experimental results in as much detail as possible. Therefore, there is no restriction on the length of the papers or the number of electronics supplementary files. For articles with computational results, the full experimental details must be provided so that the results can be reproduced. Electronic files regarding the full details of the calculation and experimental procedure, if unable to be published in a normal way, can be deposited as supplementary material (including animated pictures, videos, interactive Excel sheets, software executables and others).
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