Crowther D C, Kinghorn K J, Miranda E, Page R, Curry J A, Duthie F A I, Gubb D C, Lomas D A
Department of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Wellcome Trust/MRC Building, Hills Road, Cambridge CB2 2XY, UK.
Neuroscience. 2005;132(1):123-35. doi: 10.1016/j.neuroscience.2004.12.025.
We have developed models of Alzheimer's disease in Drosophila melanogaster by expressing the Abeta peptides that accumulate in human disease. Expression of wild-type and Arctic mutant (Glu22Gly) Abeta(1-42) peptides in Drosophila neural tissue results in intracellular Abeta accumulation followed by non-amyloid aggregates that resemble diffuse plaques. These histological changes are associated with progressive locomotor deficits and vacuolation of the brain and premature death of the flies. The severity of the neurodegeneration is proportional to the propensity of the expressed Abeta peptide to form oligomers. The fly phenotype is rescued by treatment with Congo Red that reduces Abeta aggregation in vitro. Our model demonstrates that intracellular accumulation and non-amyloid aggregates of Abeta are sufficient to cause the neurodegeneration of Alzheimer's disease. Moreover it provides a platform to dissect the pathways of neurodegeneration in Alzheimer's disease and to develop novel therapeutic interventions.
我们通过表达在人类疾病中积累的β淀粉样蛋白(Aβ)肽,在黑腹果蝇中建立了阿尔茨海默病模型。在果蝇神经组织中表达野生型和北极突变体(Glu22Gly)Aβ(1-42)肽会导致细胞内Aβ积累,随后形成类似弥漫性斑块的非淀粉样聚集体。这些组织学变化与渐进性运动功能障碍、脑空泡化以及果蝇过早死亡有关。神经退行性变的严重程度与所表达的Aβ肽形成寡聚体的倾向成正比。用刚果红处理可在体外减少Aβ聚集,从而挽救果蝇的表型。我们的模型表明,Aβ的细胞内积累和非淀粉样聚集体足以导致阿尔茨海默病的神经退行性变。此外,它为剖析阿尔茨海默病神经退行性变的途径和开发新的治疗干预措施提供了一个平台。