Kimura M, Sato M, Akatsuka A, Nozawa-Kimura S, Takahashi R, Yokoyama M, Nomura T, Katsuki M
Department of DNA Biology, School of Medicine, Tokai University, Kanagawa, Japan.
Proc Natl Acad Sci U S A. 1989 Jul;86(14):5661-5. doi: 10.1073/pnas.86.14.5661.
A minigene containing mouse cDNA coding for the smallest type of myelin basic protein and including the native promoter was constructed and used to produce transgenic shiverer mice. The hypomyelinating mouse, the shiverer, has a deletion in its myelin basic protein gene, lacks all four types of myelin basic protein in its myelin, and shows abnormal behavior such as violent tremors. Five of twenty-one transgenic shiverer mice showed recovered protein synthesis, compact myelin formation, and normal behavior. These results suggest that a single type of myelin basic protein restores myelin formation and returns the shivering phenotype to normal in the transgenic shiverer mouse.
构建了一个包含编码最小类型髓鞘碱性蛋白的小鼠cDNA并包含天然启动子的微型基因，并用于产生转基因颤抖小鼠。髓鞘形成减少的小鼠，即颤抖小鼠，其髓鞘碱性蛋白基因存在缺失，其髓鞘中缺乏所有四种类型的髓鞘碱性蛋白，并表现出诸如剧烈颤抖等异常行为。21只转基因颤抖小鼠中有5只表现出蛋白质合成恢复、致密髓鞘形成和正常行为。这些结果表明，单一类型的髓鞘碱性蛋白可恢复髓鞘形成，并使转基因颤抖小鼠的颤抖表型恢复正常。
