Pickford Fiona, Coomaraswamy Janaky, Jucker Mathias, McGowan Eileen
Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 92224, USA.
Brain Pathol. 2006 Jan;16(1):80-5. doi: 10.1111/j.1750-3639.2006.tb00564.x.
The chromosome 13 linked amyloidopathies familial British dementia (FBD) and familial Danish dementia (FDD) are caused by mutations in the C-terminus of the BRI2 gene. In both diseases, novel peptides are deposited in amyloid plaques in the brain. Several laboratories have attempted to model these diseases in BRI2 transgenic mice with limited success. While high expression levels of BRI protein were achieved in transgenic lines, no ABri-amyloidosis was observed in aged mice. This review discusses the strategies chosen and problems experienced with the development of FBD/FDD models and suggests novel approaches to model the diseases in murine models.
与13号染色体相关的淀粉样变性病，即家族性英国痴呆症（FBD）和家族性丹麦痴呆症（FDD），是由BRI2基因C端的突变引起的。在这两种疾病中，新型肽沉积在大脑的淀粉样斑块中。几个实验室试图在BRI2转基因小鼠中模拟这些疾病，但成效有限。虽然在转基因品系中实现了BRI蛋白的高表达水平，但在老年小鼠中未观察到ABri淀粉样变性。本综述讨论了在开发FBD/FDD模型时所采用的策略和遇到的问题，并提出了在小鼠模型中模拟这些疾病的新方法。
