Localization of ALADIN and its role in intracellular signal trasduction

AAA综合征（TripleA syndrome，AAA）主要发生在北非地区，表现为促肾上腺皮质激素（ACTH）抵抗性肾上腺皮质功能不全、泪溢和失弛缓症。当这种疾病中ACTH分泌充足，并且正常ACTH受体出现在肾上腺皮质时，它会发展为肾上腺功能不足。通过腺苷酸环化酶等cAMP产生系统，细胞内信号转导是不完整的。由该基因（AAAS）合成的蛋白质被命名为ALADIN（Alacrima Adrenal Insufficiency Neurorogic Disorder）或Adracalin。我建立了ALADIN的特异性抗体的制备和免疫染色，以分析该ALADIN在生物体中的分布和功能。我制备了AIADIN的C-端（386-400）和N-端（57-70）作为半抗原，并用戊二醛法偶联了每10%的牛血清白蛋白。通过ELISA证实了高抗体滴度。组织学研究表明，N端抗体的浓度为1：1500，C端抗体的浓度为1：2500。目前，通过Western blot分析ALADIN在肾上腺皮质中的蛋白表达。

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.