C型尼曼-匹克病小鼠脑内胶质细胞的变化

目的　研究C型尼曼 匹克病 (Niemann PickdiseasetypeC ,NPC)脑内胶质细胞的变化及其与神经退行性变的关系。方法　应用组织蛋白酶D(Cat D)和胶质原纤维酸性蛋白 (GFAP)抗体 ,对生后不同年龄的NPC小鼠和正常小鼠的脑组织切片进行免疫组织化学PAP法染色 ,观察小胶质细胞和星形胶质细胞在不同脑区的变化。结果　NPC小鼠脑内Cat D深染的小胶质细胞和GFAP免疫反应性星形胶质细胞的数量随着年龄增加 ,出现细胞肿胀 ,尤其在大脑皮质、丘脑、脑干和黑质等最为显著。两种胶质细胞在分布区域上重叠 ,且与神经元严重损伤、丢失的脑区相吻合。结论　NPC小鼠脑内激活的小胶质细胞和星形胶质细胞在神经退行性变过程中发挥重要作用

Changes in glial cells in the brain of mice with Niemann-Pick disease type C

Objective To study the changes in glial cells in the brain of mice with Niemann-Pick disease type C (NPC) and their relationship with neurodegeneration.Methods Brain sections from both mice with NPC and normal mice of postnatal 1, 3, 5, 8 and 11 weeks were immunostained by using antibodies against cathepsin-D (Cat-D) and glial fibrillary acid protein (GFAP) and the PAP technique. The changes in microglia and astrocytes of various brain regions were observed.Results The densely Cat-D-immunostained microglia and GFAP-immunoreactive astrocytes increased with age in number and became swollen in almost all brain regions of the NPC mice, especially in the cerebral cortex,thalamus,brain stem and substantia nigra. The regional distribution of the two types of glial cells overlapped and corresponded to that of severe injury and loss of neurons. Conclusion Activated microglia and astrocytes in the brain of NPC mice may play an important role in neurodegeneration.