Department of Biology, University of Virginia, Charlottesville, VA, USA
Department of Biology, University of Virginia, Charlottesville, VA, USA.
EMBO J. 2018 Nov 15;37(22). doi: 10.15252/embj.2018100241. Epub 2018 Oct 22.
The mechanisms of mitochondrial dysfunction in Alzheimer's disease are incompletely understood. Using two-photon fluorescence lifetime microscopy of the coenzymes, NADH and NADPH, and tracking brain oxygen metabolism with multi-parametric photoacoustic microscopy, we show that activation of lysosomal mechanistic target of rapamycin complex 1 (mTORC1) by insulin or amino acids stimulates mitochondrial activity and regulates mitochondrial DNA synthesis in neurons. Amyloid-β oligomers, which are precursors of amyloid plaques in Alzheimer's disease brain and stimulate mTORC1 protein kinase activity at the plasma membrane but not at lysosomes, block this Nutrient-induced Mitochondrial Activity (NiMA) by a mechanism dependent on tau, which forms neurofibrillary tangles in Alzheimer's disease brain. NiMA was also disrupted in fibroblasts derived from two patients with tuberous sclerosis complex, a genetic disorder that causes dysregulation of lysosomal mTORC1. Thus, lysosomal mTORC1 couples nutrient availability to mitochondrial activity and links mitochondrial dysfunction to Alzheimer's disease by a mechanism dependent on the soluble building blocks of the poorly soluble plaques and tangles.
阿尔茨海默病中线粒体功能障碍的机制尚不完全清楚。我们使用双光子荧光寿命显微镜对辅酶 NADH 和 NADPH 进行检测,并通过多参数光声显微镜跟踪大脑的氧代谢,结果表明胰岛素或氨基酸激活溶酶体雷帕霉素靶蛋白复合物 1(mTORC1)会刺激神经元中线粒体的活性并调节线粒体 DNA 的合成。淀粉样β寡聚体是阿尔茨海默病大脑中淀粉样斑块的前体,它在质膜而非溶酶体上刺激 mTORC1 蛋白激酶的活性,通过一种依赖于在阿尔茨海默病大脑中形成神经原纤维缠结的 tau 的机制来阻断这种营养诱导的线粒体活性(NiMA)。NiMA 也在两名结节性硬化症患者的成纤维细胞中被破坏,结节性硬化症是一种遗传性疾病,会导致溶酶体 mTORC1 的失调。因此,溶酶体 mTORC1 将营养物质的可用性与线粒体的活性联系起来,并通过一种依赖于这些难以溶解的斑块和缠结的可溶性组成部分的机制将线粒体功能障碍与阿尔茨海默病联系起来。