Department of Comparative Biomedical Sciences, Royal Veterinary College, London NW1 0TU, UK.
School of Natural Sciences, Institute of Structural and Molecular Biology, Birkbeck, University of London, London WC1E 7HX, UK.
Cells. 2024 Apr 4;13(7):631. doi: 10.3390/cells13070631.
Neonatal hypoxia-ischemia (HI) affects 2-3 per 1000 live births in developed countries and up to 26 per 1000 live births in developing countries. It is estimated that of the 750,000 infants experiencing a hypoxic-ischemic event during birth per year, more than 400,000 will be severely affected. As treatment options are limited, rapidly identifying new therapeutic avenues is critical, and repurposing drugs already in clinical use offers a fast-track route to clinic. One emerging avenue for therapeutic intervention in neonatal HI is to target mitochondrial dysfunction, which occurs early in the development of brain injury. Mitochondrial dynamics are particularly affected, with mitochondrial fragmentation occurring at the expense of the pro-fusion protein Optic Atrophy (OPA)1. OPA1, together with mitofusins (MFN)1/2, are required for membrane fusion, and therefore, protecting their function may also safeguard mitochondrial dynamics. Leflunomide, an FDA-approved immunosuppressant, was recently identified as an activator of MFN2 with partial effects on OPA1 expression. We, therefore, treated C17.2 cells with Leflunomide before or after oxygen-glucose deprivation, an in vitro mimic of HI, to determine its efficacy as a neuroprotection and inhibitor of mitochondrial dysfunction. Leflunomide increased baseline OPA1 but not MFN2 expression in C17.2 cells. However, Leflunomide was unable to promote cell survival following OGD. Equally, there was no obvious effect on mitochondrial morphology or bioenergetics. These data align with studies suggesting that the tissue and mitochondrial protein profile of the target cell/tissue are critical for taking advantage of the therapeutic actions of Leflunomide.
新生儿缺氧缺血性(HI)影响发达国家每 1000 例活产中 2-3 例,发展中国家每 1000 例活产中多达 26 例。据估计,每年有 75 万婴儿在分娩过程中经历缺氧缺血事件,其中超过 40 万婴儿将受到严重影响。由于治疗选择有限,因此迅速确定新的治疗途径至关重要,而重新利用已在临床使用的药物为进入临床提供了快速途径。治疗新生儿 HI 的一种新兴途径是针对线粒体功能障碍,这发生在脑损伤发展的早期。线粒体动力学受到特别影响,线粒体碎片化以牺牲促融合蛋白视神经病(OPA)1 为代价。OPA1 与线粒体融合蛋白(MFN)1/2 一起是膜融合所必需的,因此保护其功能也可能保护线粒体动力学。来氟米特是一种获得 FDA 批准的免疫抑制剂,最近被确定为 MFN2 的激活剂,对 OPA1 表达有部分作用。因此,我们在用氧葡萄糖剥夺(HI 的体外模拟)处理 C17.2 细胞之前或之后用来氟米特处理 C17.2 细胞,以确定其作为神经保护剂和线粒体功能障碍抑制剂的功效。来氟米特增加了 C17.2 细胞中的基础 OPA1 表达,但不增加 MFN2 表达。然而,来氟米特不能促进 OGD 后的细胞存活。同样,线粒体形态或生物能量学也没有明显影响。这些数据与表明靶细胞/组织的组织和线粒体蛋白谱对于利用来氟米特的治疗作用至关重要的研究一致。