Localization of ALADIN and its role in intracellular signal trasduction

AAA（三A）综合征，局限于北非地区，表现为促肾上腺皮质激素（ACTH）抵抗性肾上腺功能不全、白斑和失弛缓症。虽然在这种疾病中ACTH分泌充足，并且正常的ACTH受体出现在肾上腺皮质，但它会发展为肾上腺功能不全。虽然腺苷环化酶等cAMP生成系统，但胞内信号转导尚未完成。由该基因合成的蛋白质（AAAS）根据这种特殊的疾病状态被命名为ALADIN （Alacrima肾上腺功能不全神经紊乱）或Adracalin。建立了ALADIN特异性抗体的制备和免疫染色方法，分析该ALADIN在机体中的分布及其功能。制备了AIADIN的c -末端（386-400）和n -末端（57-70）作为半抗原，用戊二醛法偶联牛血清白蛋白。ELISA法证实抗体滴度高。组织学研究表明，n端抗体在1:15 00和c端抗体在1:25 00时可用。目前，应用Western blot方法对肾上腺皮质ALADIN蛋白的表达进行了分析。

AAA (Triple A) syndrome, which confines in north Africa district, shows adrenocorticotrophic hormone (ACTH)-resistant adrenal insufficiency insufficiency, alacrima and achalasia. While ACTH is secreted enough in this disorder, and a normal ACTH receptor appears to an adrenal cortex, it develops adrenal insuffuiency. Though cAMP generating system such as adenyl cyclase, the intracellular signal transduction is not completed. The protein synthesized by the gene (AAAS) is named ALADIN (Alacrima Adrenal Insufficiency Neurorogic Disorder) or Adracalin by this specific disease state. I established making and immunostaining of a specific antibody for ALADIN to analyze distribution in organism of this ALADIN and a function. I prepared C-terminal (386-400) and N-terminal (57-70) of AIADIN as hapten and I comjugate each tenth bovine serum albumin by glutaraldehyde method. High antibody titer was confirmed by ELISA. Histological studies relealed that N-terminal antibody can be used at 1 : 1500 and C-terminal antibody at 1 : 2500. By the present, the protein expression of ALADIN in an adrenal cortex by Western blot analysis.