Localization of ALADIN and its role in intracellular signal trasduction

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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.