Analyses of congenital lipoid adrenal hyperplasia and nuerodteroids using StAR knockout mice

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1)We generated Apolipoprotein A-1(Apo A1) and steroidogenic regulatory protein(StAP) double knockout mice (double KO). We analyzed histology of steroidogenic tissues in wild, StAR knockout(StAR KO), and double KO using oil red O staining. Results (1)In neonates, lipid deposit in adrenal gland and testis showed StAR KO>>double KO>wild. No lipid deposit in ovary was observed in any genotypes. (2)In young adult (8 week old), lipid deposit in testis and ovary showed StAR KO>>double KO>wild. These data suggested the predominant role of circulating cholesterol in the lipid accumulation by StAR deficiency.2)We investigated the protein expression of StAR and cholesterol side chain cleavage enzyme(P450soc) in brain using immunohistochemical staining. In motor cortex as well as hyppocampus, we identidied some cells expressing both StAR and P450soc. These data supported the functional significance of StAR in neurosteroidogenesis in brain.