Histopathological study on neurodegeneration in the mouse model of xeroderma pigmentosum and Cockayne's syndrome

我们对小鼠色素性干皮病和Cockayne综合征模型进行了组织病理学研究。用XPA-/-和CSB-/-成年小鼠配对获得11只新生小鼠。这11只小鼠在出生后12天进行了检查。3只小鼠的体重和脑重量均小于其他8只小鼠(平均值分别为3.79：6.09g/p=0.000l和0.26：0.32g/p=0.0005)。这3只小鼠在基因上都是XPA-/-CSB-/-。在病理学上，包括大脑、脑干、小脑和脊髓在内的中枢神经系统没有明显的异常。用抗神经丝、胶质纤维酸性蛋白、突触素、α-突触核蛋白和萎缩蛋白-1的抗体进行免疫组织化学检测，未能显示出这11只小鼠在密度和分布上的任何差异。综上所述，我们认为小脑(小头畸形)是由于缺乏XPA和CSB基因而引起的，这些基因可能在大脑发育早期控制原始细胞分裂和细胞凋亡方面发挥了一定的作用。

We carried out the histopathological investigations on the mouse model of xeroderma pigmentosum and Cockayne's syndrome. Eleven new born mice were obtaind by mating XPA-/- and CSB-/- adult mice. These 11 mice were examind at day 12 postnatal. Three mice showed smaller values of body arid brain weights than those of 8 other mice (average 3.79 : 6.09 g/p=0.000l and average 0.26 : 0.32 g/p=0.0005, respectively). These 3 mice were genetically XPA-/-CSB-/-.Hitologically, there were no remarkable abnormalities in the central nervous system, including the cerebrum, brain stem, cerebellum and spinal cord. Immunohistochemical examination, using antibodies against neurofilament, glial fibrillary acidic protein, synaptpphysin, aipha-synuclein and atrophin-1, failed to demonstrate any differences in density and distributions between thses 11 mice.In conclusion, we considered that small brain (microcephaly) without particular dysorganization resulted from the lacking of both genes XPA and CSB and that these genes may play certain important roles in controlling the primitive cell division and apoptosis in the early developmental phase of the brain.In parallel with the above investigation, we performed electron immunohisto-chemical study on the two isoform of synaphin/complexin in the rat central nervous system, revealing the different distribution pattern between them.