Graduate School of Biosphere Science, Hiroshima University, 1-7-1 Kagamiyama, Higashi-hiroshima 739-8521, Japan.
Neurosci Lett. 2010 Jan 22;469(2):273-7. doi: 10.1016/j.neulet.2009.12.015. Epub 2009 Dec 16.
Amyloid precursor protein (APP) is strongly related to the onset of Alzheimer's disease. It possesses cleavage sites for beta- and gamma-secretases, and the resulting cleaved products (amyloid-beta peptides) are capable of causing neurotoxicity. Such cleavage is promoted by the Swedish and London mutations (APPSwe/Lon) inside the APP gene. Here, we characterized APPSL transgenic mice (APPSL-Tg) to determine the effects of this mutation. We observed that both the amount of insoluble amyloid-beta and the ratio of amyloid-beta 42/40 increased promptly in the brain during 6-16 months of age. Amyloid-beta plaques were observed in whole brain sections at 12 months. In contrast, the spatial memory assessed by the Morris water maze task was already impaired at 3 months, which suggested that the APPSL-Tg mice may represent an early-onset model of familial Alzheimer's disease. Furthermore, the levels of LAMP-1, a marker protein of lysosome, increased in the brain at 28 months. Such LAMP-1 protein was detected around the amyloid-beta plaques at the hippocampal regions of the APPSL-Tg mice. Our results suggested that the increase in LAMP-1 was enhanced by the accumulation of amyloid-beta occurring during aging. Our findings coincided with the pathological hallmarks of Alzheimer's disease.
淀粉样前体蛋白(APP)与阿尔茨海默病的发病密切相关。它具有β-和γ-分泌酶的切割位点,而产生的切割产物(淀粉样β肽)能够引起神经毒性。这种切割受 APP 基因内的瑞典和伦敦突变(APPSwe/Lon)的促进。在这里,我们对 APPSL 转基因小鼠(APPSL-Tg)进行了特征描述,以确定该突变的影响。我们观察到,在 6-16 个月龄期间,脑内不溶性淀粉样β的量和淀粉样β 42/40 的比例迅速增加。在 12 个月时观察到全脑切片中的淀粉样β斑块。相比之下,通过 Morris 水迷宫任务评估的空间记忆在 3 个月时已经受损,这表明 APPSL-Tg 小鼠可能代表家族性阿尔茨海默病的早发模型。此外,在 28 个月时,脑内溶酶体的标记蛋白 LAMP-1 的水平增加。在 APPSL-Tg 小鼠的海马区域,在淀粉样β斑块周围检测到这种 LAMP-1 蛋白。我们的结果表明,LAMP-1 的增加是由衰老过程中淀粉样β的积累所增强的。我们的发现与阿尔茨海默病的病理特征一致。