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This gene encodes a protein that contains a DNA-binding domain. The encoded protein acts as a dominant-negative regulator of transcription which is mediated by the retinoic acid receptor. This protein also functions as an anti-testis gene by acting antagonistically to Sry. Mutations in this gene result in both X-linked congenital adrenal hypoplasia and hypogonadotropic hypogonadism.
adrenal hypoplasia, congenital homolog; AHC; Ahch; AHX; DAX; DAX 1; Dax1; DAX-1; DSS; DSS-AHC critical region on the X chromosome protein 1; GTD; HHG; Nr0b1; NROB1; nuclear hormone receptor; nuclear receptor DAX-1; Nuclear receptor subfamily 0 group B member 1; nuclear receptor subfamily 0, group B, member 1; orphan nuclear receptor DAX-1; R0B1; SRXY2
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