Department of Cell Death and Proliferation, IIBB-CSIC, Barcelona 08036, Spain.
Hum Mol Genet. 2013 Sep 1;22(17):3460-76. doi: 10.1093/hmg/ddt201. Epub 2013 May 6.
Current evidence indicates that excess brain cholesterol regulates amyloid-β (Aβ) deposition, which in turn can regulate cholesterol homeostasis. Moreover, Aβ neurotoxicity is potentiated, in part, by mitochondrial glutathione (mGSH) depletion. To better understand the relationship between alterations in cholesterol homeostasis and Alzheimer's disease (AD), we generated a triple transgenic mice featuring sterol regulatory element-binding protein-2 (SREBP-2) overexpression in combination with APPswe/PS1ΔE9 mutations (APP/PS1) to examine key biochemical and functional characteristics of AD. Unlike APP/PS1 mice, APP/PS1/SREBP-2 mice exhibited early mitochondrial cholesterol loading and mGSH depletion. Moreover, β-secretase activation and Aβ accumulation, correlating with oxidative damage and neuroinflammation, were accelerated in APP/PS1/SREBP-2 mice compared with APP/PS1 mice. Triple transgenic mice displayed increased synaptotoxicity reflected by loss of synaptophysin and neuronal death, resulting in early object-recognition memory impairment associated with deficits in spatial memory. Interestingly, tau pathology was present in APP/PS1/SREBP-2 mice, manifested by increased tau hyperphosphorylation and cleavage, activation of tau kinases and neurofibrillary tangle (NFT) formation without expression of mutated tau. Importantly, in vivo treatment with the cell permeable GSH ethyl ester, which restored mGSH levels in APP/PS1/SREBP-2 mice, partially prevented the activation of tau kinases, reduced abnormal tau aggregation and Aβ deposition, resulting in attenuated synaptic degeneration. Taken together, these results show that cholesterol-mediated mGSH depletion is a key event in AD progression, accelerating the onset of key neuropathological hallmarks of the disease. Thus, therapeutic approaches to recover mGSH may represent a relevant strategy in the treatment of AD.
目前的证据表明,大脑胆固醇过多会调节淀粉样蛋白-β(Aβ)的沉积,而后者又可以调节胆固醇的动态平衡。此外,Aβ的神经毒性部分是由于线粒体谷胱甘肽(mGSH)耗竭而增强的。为了更好地理解胆固醇动态平衡的改变与阿尔茨海默病(AD)之间的关系,我们生成了一种三重转基因小鼠,其特征是固醇调节元件结合蛋白-2(SREBP-2)过表达与 APPswe/PS1ΔE9 突变(APP/PS1)相结合,以检查 AD 的关键生化和功能特征。与 APP/PS1 小鼠不同,APP/PS1/SREBP-2 小鼠表现出早期的线粒体胆固醇负荷和 mGSH 耗竭。此外,与 APP/PS1 小鼠相比,β-分泌酶的激活和 Aβ的积累,与氧化损伤和神经炎症相关,在 APP/PS1/SREBP-2 小鼠中加速。三重转基因小鼠表现出突触毒性增加,反映为突触小泡蛋白丢失和神经元死亡,导致与空间记忆缺陷相关的早期物体识别记忆障碍。有趣的是,在 APP/PS1/SREBP-2 小鼠中存在 tau 病理学,表现为 tau 过度磷酸化和裂解增加、tau 激酶激活和神经原纤维缠结(NFT)形成,而没有突变 tau 的表达。重要的是,体内给予可穿透细胞的 GSH 乙酯治疗,可恢复 APP/PS1/SREBP-2 小鼠中的 mGSH 水平,部分阻止 tau 激酶的激活,减少异常 tau 聚集和 Aβ沉积,从而减轻突触退化。总之,这些结果表明,胆固醇介导的 mGSH 耗竭是 AD 进展的关键事件,加速了疾病关键神经病理学特征的出现。因此,恢复 mGSH 的治疗方法可能代表 AD 治疗的一种相关策略。
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