Wang Xin-Xin, Li Mei, Xu Xiao-Wen, Zhao Wen-Bin, Jin Yi-Ming, Li Li-Li, Qin Zheng-Hong, Sheng Rui, Ni Hong
Department of Brain Research, Institute of Pediatric Research, Children's Hospital of Soochow University, Suzhou, 215025, China.
Department of Pharmacology and Laboratory of Aging and Nervous Diseases, Soochow University School of Pharmaceutical Science, Suzhou, 199 Ren Ai Road, Suzhou, 215123, China.
Acta Pharmacol Sin. 2025 Jan;46(1):33-51. doi: 10.1038/s41401-024-01365-x. Epub 2024 Aug 23.
As a major contributor to neonatal death and neurological sequelae, hypoxic-ischemic encephalopathy (HIE) lacks a viable medication for treatment. Oxidative stress induced by hypoxic-ischemic brain damage (HIBD) predisposes neurons to ferroptosis due to the fact that neonates accumulate high levels of polyunsaturated fatty acids for their brain developmental needs but their antioxidant capacity is immature. Ferroptosis is a form of cell death caused by excessive accumulation of iron-dependent lipid peroxidation and is closely associated with mitochondria. Mitophagy is a type of mitochondrial quality control mechanism that degrades damaged mitochondria and maintains cellular homeostasis. In this study we employed mitophagy agonists and inhibitors to explore the mechanisms by which mitophagy exerted ferroptosis resistance in a neonatal rat HIE model. Seven-days-old neonatal rats were subjected to ligation of the right common carotid artery, followed by exposure to hypoxia for 2 h. The neonatal rats were treated with a mitophagy activator Tat-SPK2 peptide (0.5, 1 mg/kg, i.p.) 1 h before hypoxia, or in combination with mitochondrial division inhibitor-1 (Mdivi-1, 20 mg/kg, i.p.), and ferroptosis inhibitor Ferrostatin-1 (Fer-1) (2 mg/kg, i.p.) at the end of the hypoxia period. The regulation of ferroptosis by mitophagy was also investigated in primary cortical neurons or PC12 cells in vitro subjected to 4 or 6 h of OGD followed by 24 h of reperfusion. We showed that HIBD induced mitochondrial damage, ROS overproduction, intracellular iron accumulation, lipid peroxidation and ferroptosis, which were significantly reduced by the pretreatment with Tat-SPK2 peptide, and aggravated by the treatment with Mdivi-1 or BNIP3 knockdown. Ferroptosis inhibitors Fer-1 and deferoxamine B (DFO) reversed the accumulation of iron and lipid peroxides caused by Mdivi-1, hence reducing ferroptosis triggered by HI. We demonstrated that Tat-SPK2 peptide-activated BNIP3-mediated mitophagy did not alleviate neuronal ferroptosis through the GPX4-GSH pathway. BNIP3-mediated mitophagy drove the P62-KEAP1-NRF2 pathway, which conferred ferroptosis resistance by maintaining iron and redox homeostasis via the regulation of FTH1, HO-1, and DHODH/FSP1-CoQ10-NADH. This study may provide a new perspective and a therapeutic drug for the treatment of neonatal HIE.
作为新生儿死亡和神经后遗症的主要原因,缺氧缺血性脑病(HIE)缺乏有效的治疗药物。缺氧缺血性脑损伤(HIBD)诱导的氧化应激使神经元易发生铁死亡,因为新生儿为满足大脑发育需求会积累高水平的多不饱和脂肪酸,但其抗氧化能力不成熟。铁死亡是一种由铁依赖性脂质过氧化过度积累引起的细胞死亡形式,与线粒体密切相关。线粒体自噬是一种线粒体质量控制机制,可降解受损线粒体并维持细胞内稳态。在本研究中,我们使用线粒体自噬激动剂和抑制剂来探索线粒体自噬在新生大鼠HIE模型中发挥抗铁死亡作用的机制。将7日龄新生大鼠右侧颈总动脉结扎,然后暴露于缺氧环境2小时。在缺氧前1小时,用线粒体自噬激活剂Tat-SPK2肽(0.5、1mg/kg,腹腔注射)对新生大鼠进行治疗,或与线粒体分裂抑制剂-1(Mdivi-1,20mg/kg,腹腔注射)联合使用,并在缺氧期结束时使用铁死亡抑制剂Ferrostatin-1(Fer-1)(2mg/kg,腹腔注射)。还在体外对原代皮质神经元或PC12细胞进行4或6小时的氧糖剥夺,然后再灌注24小时,研究线粒体自噬对铁死亡的调节作用。我们发现,HIBD诱导线粒体损伤、活性氧过度产生、细胞内铁积累、脂质过氧化和铁死亡,用Tat-SPK2肽预处理可显著降低这些指标,而用Mdivi-1处理或敲低BNIP3则会加重这些指标。铁死亡抑制剂Fer-1和去铁胺B(DFO)可逆转Mdivi-1引起的铁和脂质过氧化物积累,从而减少HI引发的铁死亡。我们证明,Tat-SPK2肽激活的BNIP3介导的线粒体自噬并非通过GPX4-GSH途径减轻神经元铁死亡。BNIP3介导的线粒体自噬驱动P62-KEAP1-NRF2途径,该途径通过调节FTH1、HO-1和DHODH/FSP1-CoQ10-NADH来维持铁和氧化还原稳态,从而赋予抗铁死亡能力。本研究可能为新生儿HIE的治疗提供新的视角和治疗药物。