Waddles小鼠遗传性小脑性共济失调的CAR8蛋白表达

Study on the expression of CAR8 in Waddles Mice with Hereditary Cerebellar Ataxia

目的探讨Waddles（Wdl）小鼠遗传性小脑性共济失调的CAR8蛋白表达的分子机制。方法采用成年2—4个月的Wdl小鼠30只和同等数量同胎生的正常小鼠进行试验。使用亲和法提纯的家兔的CAR8蛋白多克隆抗体进行Western blot分析。结果在正常和＋／Wdl小鼠的小脑细胞中可见CAR8蛋白的表达且表达量相似，而在Wdl小鼠的小脑细胞中不能探查到CAR8蛋白的表达。在正常、杂合子及Wdl小鼠，IP3R1蛋白表达无明显差别。结论Wdl小鼠CAR8基因突变改变其基因的蛋白表达，出现蛋白质翻译障碍。

Objective To investigate the molecular mechanism of the expression of CAR8 protein in waddles mice with hereditary eerebellar ataxia,Methods 30 Aduh（2 - 4 months）Wdl mice and 30 normal littermates were used for quantitative analyses. Westem blotting examination was used to investigate whether the expression of CAR8 protein at translational level was affected by the mutated Car8 gene , Results The expression of Car8 protein was not detected in Wdl mice, while the level of protein in homozygous, normal and heterozygous mice was similar. The expression of IP3R1 was not affected by the mutation of Car8 gene in normal, heterozygous and Wdl mice, Conclusion The mutated Car8 gene in Wdl mice reduces its protein expression at translational levels.