Hum Mol Genet. 2013 Oct 1;22(19):3931-46. doi: 10.1093/hmg/ddt247. Epub 2013 Jun 4.
Bioenergetic dysfunction occurs in Alzheimer's disease (AD) and mild cognitive impairment (MCI), a clinical syndrome that frequently precedes symptomatic AD. In this study, we modeled AD and MCI bioenergetic dysfunction by transferring mitochondria from MCI, AD and control subject platelets to mtDNA-depleted SH-SY5Y cells. Bioenergetic fluxes and bioenergetics-related infrastructures were characterized in the resulting cytoplasmic hybrid (cybrid) cell lines. Relative to control cybrids, AD and MCI cybrids showed changes in oxygen consumption, respiratory coupling and glucose utilization. AD and MCI cybrids had higher ADP/ATP and lower NAD+/NADH ratios. AD and MCI cybrids exhibited differences in proteins that monitor, respond to or regulate cell bioenergetic fluxes including HIF1α, PGC1α, SIRT1, AMPK, p38 MAPK and mTOR. Several endpoints suggested mitochondrial mass increased in the AD cybrid group and probably to a lesser extent in the MCI cybrid group, and that the mitochondrial fission-fusion balance shifted towards increased fission in the AD and MCI cybrids. As many of the changes we observed in AD and MCI cybrid models are also seen in AD subject brains, we conclude reduced bioenergetic function is present during very early AD, is not brain-limited and induces protean retrograde responses that likely have both adaptive and mal-adaptive consequences.
生物能量功能障碍发生在阿尔茨海默病(AD)和轻度认知障碍(MCI)中,这是一种经常发生在有症状的 AD 之前的临床综合征。在这项研究中,我们通过将 MCI、AD 和对照受试者血小板中的线粒体转移到 mtDNA 耗尽的 SH-SY5Y 细胞中,模拟了 AD 和 MCI 的生物能量功能障碍。我们对由此产生的细胞质杂种(cybrid)细胞系中的生物能量通量和与生物能量相关的基础设施进行了特征描述。与对照 cybrid 相比,AD 和 MCI cybrid 显示出氧消耗、呼吸偶联和葡萄糖利用的变化。AD 和 MCI cybrid 具有更高的 ADP/ATP 和更低的 NAD+/NADH 比值。AD 和 MCI cybrid 表现出差异的蛋白,这些蛋白可以监测、响应或调节细胞生物能量通量,包括 HIF1α、PGC1α、SIRT1、AMPK、p38 MAPK 和 mTOR。有几个终点表明 AD cybrid 组中线粒体质量增加,而 MCI cybrid 组可能增加较少,并且 AD 和 MCI cybrid 中线粒体的分裂-融合平衡向分裂增加的方向转移。由于我们在 AD 和 MCI cybrid 模型中观察到的许多变化也出现在 AD 患者的大脑中,因此我们得出结论,在早期 AD 期间存在生物能量功能降低,并且不受大脑限制,并诱导多态性逆行反应,这些反应可能具有适应性和不良适应性后果。