Reincorporation of cathepsin D in neurons of mouse brain.

中枢神经系统特异性组织蛋白D(Cd)缺陷小鼠(Cd：NesCre)在P40附近癫痫发作，其表型类似于神经元蜡样脂褐素沉积症。免疫组织化学方法检测到Cd：NesCre小鼠的神经元中有CD蛋白表达，但未检测到Cd基因的表达。这些结果表明，Cd：NesCre神经元接受来自其他类型细胞的Cd。我们还建立了检测溶酶体酶转运系统的雏鸡实验模型，建立了转基因进入雏鸡基因组的稳定表达系统。

Central nervous system specific cathepsin D (CD) deficient mice (CD:NesCre) ied around P40 by seizures and their phenotype was similar to neuronal ceroid lipofuscinosis. We noticed that CD protein was detected in neurons of CD:NesCre mice by immunohistochemistry, although CD mRNA was not expressed in the neurons. These results indicate that CD:NesCre neurons receive CD from other type of cells. We have also developed the chick experimental model to examine the transport system of lysosomal enzymes and established the stable expression system of transgenes into chick genome.