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

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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.