The pathogenesis of congenital lipoid adrenal hyperplasia in gonads revealed by StAR knockout mice

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I generated steroidogenic acute regulatory protein (StAR) knockout mice (StAR KO) using gene targeting. I crossed StAR KO mice and naturally occurred gonadotropin-releasiug hormone (GnRH) gene abnormal mice to generate StAR/GnRH double mutant mice (StAR/GnRH double KO). I investigated gonadal histology in StAR KO and StAR/GnRH double KO from birth to 8 weeks.1. at birth : (1) Testes in StAR KO had mild lipid deposits in interstitial space. Ovaries did not. (2) Both testes and ovaries in StAR/GnRH double KO did not have any Iipid deposits.2. from birth to 6 weeks : (1) Both testes and ovaries in StAR KO did not have massive lipid deposits. (2) Both testes and ovaries in StAR/GnRH double KO did not have any lipid deposits.3. 8 weeks : (1) Both testes and ovaries in StAR/GnRH double KO were hypoplastic compared to StAR KO. (2) Both testes and ovaries in StAR KO had massive lipid deposits. (3) Both testes and ovaries in StAR/GnRH double KO lacked lipid deposits. (4) Exogenous gonadotoropin treatment in StAR/GnRH double KO induced lipid deposits in both testes and ovaries.These data strongly indicated that lipid deposits in StAR KO gonads after pubertal age is gonadotoropin dependent.