Laboratory of Molecular and Cellular Neuroscience, The Rockefeller University, 1230 York Ave., New York, NY 10021, USA.
FASEB J. 2012 Dec;26(12):5115-23. doi: 10.1096/fj.12-212985. Epub 2012 Sep 12.
The pathways leading specifically to the toxic Aβ42 peptide production, a key event in Alzheimer's disease (AD), are unknown. While searching for pathways that mediate pathological increases of Aβ42, we identified Aftin-4, a new compound that selectively and potently increases Aβ42 compared to DMSO (N2a cells: 7-fold; primary neurons: 4-fold; brain lysates: 2-fold) with an EC(50) of 30 μM. These results were confirmed by ELISA and IP-WB. Using affinity chromatography and mass spectrometry, we identified 3 proteins (VDAC1, prohibitin, and mitofilin) relevant to AD that interact with Aftin-4, but not with a structurally similar but inactive molecule. Electron microscopy studies demonstrated that Aftin-4 induces a reversible mitochondrial phenotype reminiscent of the one observed in AD brains. Sucrose gradient fractionation showed that Aftin-4 perturbs the subcellular localization of γ-secretase components and could, therefore, modify γ-secretase specificity by locally altering its membrane environment. Remarkably, Aftin-4 shares all these properties with two other "AD accelerator" compounds. In summary, treatment with three Aβ42 raising agents induced similar biochemical alterations that lead to comparable cellular phenotypes in vitro, suggesting a common mechanism of action involving three structural cellular targets.
具体导致阿尔茨海默病(AD)中关键事件——毒性 Aβ42 肽产生的途径尚不清楚。在寻找介导 Aβ42 病理性增加的途径时,我们鉴定了 Aftin-4,这是一种新的化合物,与 DMSO 相比,它能选择性且有效地增加 Aβ42(N2a 细胞:7 倍;原代神经元:4 倍;脑裂解物:2 倍),EC50 为 30 μM。这些结果通过 ELISA 和 IP-WB 得到了证实。通过亲和层析和质谱分析,我们鉴定出与 AD 相关的 3 种蛋白(VDAC1、 prohibitin 和 mitofilin)与 Aftin-4 相互作用,但与结构相似但无活性的分子不相互作用。电子显微镜研究表明,Aftin-4 诱导出一种与 AD 大脑中观察到的类似的可逆线粒体表型。蔗糖梯度分级显示,Aftin-4 扰乱了 γ-分泌酶成分的亚细胞定位,因此可以通过局部改变其膜环境来改变 γ-分泌酶的特异性。值得注意的是,Aftin-4 与另外两种“AD 加速剂”化合物具有所有这些特性。总之,三种 Aβ42 升高剂的处理诱导了相似的生化改变,导致体外具有相似的细胞表型,这表明涉及三个结构细胞靶标的共同作用机制。