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In this study, we show that Dlx5\/6 are expressed in the fetal Leydig cells during testis development. We examined the effect of Dlx5 expression on the promoter activation of the steroidogenic acute regulatory protein (StAR) gene, which is essential for gonadal and adrenal steroidogenesis, in a Leydig cell line. Dlx5 efficiently activates the StAR promoter when GATA-4, another transcription factor essential for testicular steroidogenesis, was coexpressed. The transcriptional activation required the GATA-4-recognition element in the StAR promoter region and Dlx5 can physically interact with GATA-4. Furthermore, we herein show that the double inactivation of Dlx5 and Dlx6 in the mouse leads to decreased testosterone level and abnormal masculinization phenotype. These results suggest that Dlx5 and Dlx6 participate in the control of steroidogenesis during testis development. The findings of this study may open the way to analyze human congenital birth defects.<\/jats:p>","DOI":"10.1210\/en.2007-1265","type":"journal-article","created":{"date-parts":[[2008,2,15]],"date-time":"2008-02-15T01:39:43Z","timestamp":1203039583000},"page":"2090-2097","source":"Crossref","is-referenced-by-count":47,"title":["Positive Regulation of Steroidogenic Acute Regulatory Protein Gene Expression through the Interaction between Dlx and GATA-4 for Testicular Steroidogenesis"],"prefix":"10.1210","volume":"149","author":[{"given":"Hisayo","family":"Nishida","sequence":"first","affiliation":[{"name":"Center for Animal Resources and Development (H.N., S.M., Y.O., K.S., N.N., G.Y.), Graduate School of Medical and Pharmaceutical Sciences and the Global COE Research Program, Kumamoto University, Kumamoto 860-0811, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichi","family":"Miyagawa","sequence":"first","affiliation":[{"name":"Center for Animal Resources 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