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

我们对着色性干皮病和Cockayne综合征小鼠模型进行了组织病理学研究。将XPA-/-和CSB-/-成年小鼠交配获得11只新生小鼠。这11只小鼠在出生后第12天进行检查。3只小鼠的体重和脑重量值小于其他8只小鼠（平均值分别为3.79：6.09 g/p= 0.000 l和0.26：0.32 g/p=0.0005）。这3只小鼠的遗传学为XPA-/-CSB-/-。组织学检查显示，中枢神经系统（包括大脑、脑干、小脑和脊髓）无明显异常。使用针对神经丝、神经胶质细胞酸性蛋白、突触素、α-突触核蛋白和萎缩蛋白-1的抗体的免疫组织化学检查未能证明这11只小鼠之间的密度和分布的任何差异。我们认为小的大脑（小头畸形）XPA和CSB基因的缺失可能是导致组织结构异常的主要原因，这两个基因可能在控制组织结构异常中起重要作用。与此同时，我们对大鼠中枢神经系统突触素/复合素的两种异构体进行了电镜化学研究，揭示了它们在中枢神经系统中的不同分布模式。

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.