Localization of ALADIN and its role in intracellular signal trasduction

AAA(AAA)综合征局限于北非地区，表现为肾上腺皮质激素(ACTH)抵抗的肾上腺功能不全、软化和失弛缓症。当ACTH在这种疾病中分泌足够多，正常的ACTH受体出现在肾上腺皮质时，就会出现肾上腺功能不全。虽然cAMP产生系统如腺苷环化酶，但细胞内的信号转导还没有完成。由该基因合成的蛋白质(AAAS)被这种特定的疾病状态命名为Aladin(Alacrima Adrenal Infficiency Neurorogic Disorder)或Adracalin。我建立了一种针对阿拉丁的特异性抗体的制备和免疫染色，以分析该阿拉丁在机体中的分布和功能。我制备了AIADIN的C-端(386-400)和N-端(57-70)作为半抗原，并用戊二醛法制备了每10个牛血清白蛋白。经酶联免疫吸附试验证实抗体效价高。组织学研究表明，N-端抗体可用于1：1500，C-端抗体可用于1：2500。目前，通过Western印迹分析，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.