中文摘要：

目的检测Wnt抑制因子-1（Wif-1）在成年肌萎缩脊髓侧索硬化症（ALS）转基因模型小鼠脊髓内的表达变化，进一步探讨Wif-1在ALS发病中的作用。方法选取ALS转基因鼠和同窝野生型鼠各54只，分别于鼠龄95d、108d、122d取材，部分鼠经4％多聚甲醛心脏灌注固定、分离脊髓、制备冷冻切片，应用免疫荧光染色技术检测脊髓内Wif-1的分布及变化；部分鼠取出脊髓、匀浆，应用RT—PCR和Westernblotting方法观察不同时间点Wif-1mRNA及其蛋白表达水平变化。结果成年ALS转基因鼠和同窝野生型鼠脊髓的中央管、灰质、白质中均可检测到Wif-1阳性细胞，在灰质内，Wif-1阳性细胞主要分布于脊髓前角；在白质内，神经元的轴突呈阳性反应。与同窝野生型鼠比较，ALS转基因鼠Wif-1阳性细胞显著增多，Wif-1mRNA和蛋白表达在95d变化不明显，在108d、122d均有升高，差异具有统计学意义（P〈0．05），其中108d升高最明显。结论在ALS转基因鼠发病过程中Wif-1阳性细胞明显增多，Wif-1mRNA和蛋白表达升高，表明Wif—1与ALS的发生密切相关。

英文摘要：

Objective To explore the change of Wnt inhibitor factor-1 （Wif-1） expression in the spinal cord of the adult amyotrophic lateral sclerosis （ALS） transgenic mice,in order to investigate the function of Wif-1 in the pathogenesis of ALS. Methods Some ALS transgenic mice and wild type mice were anesthetized deeply and then perfused intracardially with 4% paraformaldehyde at 95-days, 108-days and 122-days. The spinal cords were separated and sectioned. The distribution and change of Wif-1 in the spinal cords were detected by immunofluorescence labeling technology. Some animals were killed and the spinal cords were separated at 95-days, 108-days and 122-days. The expression of Wif-1 mRNA and Wif-1 protein in the spinal cords were detected using reverse transcription polymerase chain reaction （RT-PCR） and Western blotting techniques. Results Wif-1 positive cells were detected in the central canal, gray matter and white matter of the adult spinal cord in ALS transgenic mice and wild type mice. Within the gray matter, most of the positive cells were located in the ventral horn. In the white matter, the positive reaction was detected in the neuron axon. Compared with the wild type mice, the Wif-1 positive cells were increased significantly in the ALS transgenic mice, the expression of Wif-1 mRNA and Wif-1 protein were increased at 108-days and 122-days, there were significant differences （P 〈 0. 05 ）, especially at 108-days. At 95-days, there was no significant difference. Conclusion Wif-1 positive cells and Wif-I mRNA and protein expression were all significantly increased in the pathogenesis of ALS transgenic mice. The expression of Wif-1 was closely related to the pathogenesis of ALS.