Yin Jun Xiang, Maalouf Marwan, Han Pengcheng, Zhao Minglei, Gao Ming, Dharshaun Turner, Ryan Christopher, Whitelegge Julian, Wu Jie, Eisenberg David, Reiman Eric M, Schweizer Felix E, Shi Jiong
Department of Neurology, Barrow Neurological Institute, St. Joseph Hospital and Medical Center, Phoenix, AZ, USA.
Department of Biological Chemistry, Molecular Biology Institute, Howard Hughes Medical Institute, UCLA-DOE Institute for Genomics and Proteomics, University of California, Los Angeles, CA, USA.
Neurobiol Aging. 2016 Mar;39:25-37. doi: 10.1016/j.neurobiolaging.2015.11.018. Epub 2015 Dec 7.
Sporadic Alzheimer's disease (AD) is responsible for 60%-80% of dementia cases, and the most opportune time for preventive intervention is in the earliest stage of its preclinical phase. As traditional mitochondrial energy substrates, ketone bodies (ketones, for short), beta-hydroxybutyrate, and acetoacetate, have been reported to provide symptomatic improvement and disease-modifying activity in epilepsy and neurodegenerative disorders. Recently, ketones are thought as more than just metabolites and also as endogenous factors protecting against AD. In this study, we discovered a novel neuroprotective mechanism of ketones in which they blocked amyloid-β 42, a pathologic hallmark protein of AD, entry into neurons. The suppression of intracellular amyloid-β 42 accumulation rescued mitochondrial complex I activity, reduced oxidative stress, and improved synaptic plasticity. Most importantly, we show that peripheral administration of ketones significantly reduced amyloid burden and greatly improved learning and memory ability in a symptomatic mouse model of AD. These observations provide us insights to understand and to establish a novel therapeutic use of ketones in AD prevention.
散发性阿尔茨海默病(AD)占痴呆病例的60%-80%,预防干预的最佳时机是在其临床前期的最早阶段。作为传统的线粒体能量底物,酮体(简称酮)、β-羟基丁酸酯和乙酰乙酸已被报道在癫痫和神经退行性疾病中具有症状改善和疾病修饰活性。最近,酮不仅被视为代谢产物,还被视为预防AD的内源性因子。在本研究中,我们发现了酮的一种新的神经保护机制,即它们阻止了AD的病理标志性蛋白淀粉样β蛋白42进入神经元。细胞内淀粉样β蛋白42积累的抑制挽救了线粒体复合体I的活性,降低了氧化应激,并改善了突触可塑性。最重要的是,我们表明,在有症状的AD小鼠模型中,外周给予酮显著降低了淀粉样蛋白负荷,并大大改善了学习和记忆能力。这些观察结果为我们理解和建立酮在AD预防中的新治疗用途提供了见解。