Borchelt D R, Wong P C, Sisodia S S, Price D L
Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Brain Pathol. 1998 Oct;8(4):735-57. doi: 10.1111/j.1750-3639.1998.tb00198.x.
Over the past several years, there has been enormous progress in generating transgenic mice that model aspects of human neurodegenerative diseases. These studies build upon the efforts of molecular geneticists who have identified a number of genes that, when mutated, cause familial forms of these diseases. In this review, we focus on the mutations that cause familial forms of Alzheimer's disease (AD) and amyotrophic lateral sclerosis (ALS), and transgenic mouse models that develop clinical and pathological abnormalities resembling those occurring in the human diseases.
在过去几年中,在培育模拟人类神经退行性疾病某些方面的转基因小鼠方面取得了巨大进展。这些研究建立在分子遗传学家的努力之上,他们已经鉴定出一些基因,这些基因发生突变时会导致这些疾病的家族形式。在这篇综述中,我们重点关注导致阿尔茨海默病(AD)和肌萎缩侧索硬化症(ALS)家族形式的突变,以及出现类似于人类疾病中发生的临床和病理异常的转基因小鼠模型。