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使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治疗。

Gene Therapy for Parkinson's Disease Using Midbrain Developmental Genes to Regulate Dopaminergic Neuronal Maintenance.

机构信息

Channelopathy Research Center (CRC), Dongguk University College of Medicine, 32 Dongguk-ro, Goyang 10326, Republic of Korea.

Department of Premedicine, College of Medicine, Hanyang University, FTC12, 222 Wangsimni-ro, Seoul 04763, Republic of Korea.

出版信息

Int J Mol Sci. 2024 Nov 18;25(22):12369. doi: 10.3390/ijms252212369.

Abstract

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.

摘要

帕金森病(PD)是第二大常见的神经退行性疾病。其特征是黑质中多巴胺能(DAnergic)神经元的进行性丧失和多巴胺(DA)水平的降低,导致运动和非运动症状。传统的 PD 治疗旨在缓解症状,但不能延缓疾病进展。PD 基因治疗为改善现有治疗方法提供了一种有前途的方法,有可能缓解显著的 PD 症状,并比传统疗法产生更少的不良反应。DA 替代方法和 DA 酶表达并不能减缓疾病进展。然而,DA 替代基因治疗,如腺相关病毒(AAV)-谷氨酸脱羧酶(GAD)和 L-氨基酸脱羧酶(AADC)基因治疗,可增加 DA 递质水平,已在早期临床试验中证明是安全有效的。疾病修饰策略,旨在减缓疾病进展,似乎很有效。这些策略包括针对下游途径、神经营养因子和中脑 DAnergic 神经元因子的治疗方法,所有这些方法在临床前和临床试验中都显示出了潜力。这些方法侧重于维持 DAnergic 神经元的完整性,而不仅仅是针对 DA 递质水平本身。特别是,关键的中脑发育和维持因子,如 Nurr1 和 Foxa2,可以与邻近的神经胶质细胞以旁分泌的方式协同作用,以保护 DAnergic 神经元免受各种毒性因素的侵害。通过用其他候选基因治疗靶向 DAnergic 神经元和神经胶质细胞,也可以达到类似的结果,但需要深入研究。神经营养因子,如神经生长因子、胶质细胞源性神经营养因子(GDNF)、脑源性神经营养因子(BDNF)和血管内皮生长因子(VEGF),也在研究其支持 DAnergic 神经元存活的潜力。此外,靶向关键下游途径的基因治疗,如自噬溶酶体途径、线粒体功能和内质网(ER)应激,也提供了有前途的途径。基因编辑和递送技术不断发展,为 PD 的有效基因治疗提供了新的机会。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/edaa/11594980/65a1448456bf/ijms-25-12369-g001.jpg

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