DAX-1, an ‘antitestis’gene

@article{Goodfellow1999DAX1A,
  title={DAX-1, an ‘antitestis’gene},
  author={P. Goodfellow and G. Camerino},
  journal={Cellular and Molecular Life Sciences CMLS},
  year={1999},
  volume={55},
  pages={857-863}
}
Abstract. The DAX-1 gene has been involved in the dosage sensitive sex reversal (DSS) phenotype, a male-to-female sex-reversal syndrome due to the duplication of a small region of human chromosome Xp21. Dax-1 and Sry have been shown to act antagonistically in the mouse system, where increasing expression of the former leads to female development and increasing activity of the latter to male development. Although these data strongly implicate DAX-1 in sex determination, the mouse and human… Expand
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Molecular mechanisms of DAX1 action.
TLDR
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Gene dosage of DAX-1, determining in sexual differentiation: duplication of DAX-1 in two sisters with gonadal dysgenesis
TLDR
This is the first report of 46,XY sex reversal in two siblings who have a maternally inherited duplication of DAX-1 associated with reduced levels of expression of downstream genes as SOX9–SF1. Expand
Expression of Dax-1 during gonadal development of the frog.
TLDR
The Dax-1 transcription declined gradually as ovarian development proceeded, but its expression was down-regulated and then up-regulated rapidly when female-to-male sex reversal was caused by administration of testosterone into female tadpoles. Expand
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TLDR
The past and present milestones in DAX-1 research are reviewed and try to provide hints about the development and fields of application of DAx-1-targeted drugs in the future. Expand
Sox3 Is Required for Gonadal Function, but Not Sex Determination, in Males and Females
TLDR
It is concluded that Sox3 is not required for gonadal determination but is important for normal oocyte development and male testis differentiation and gametogenesis and targeted mutagenesis to delete Sox3 from mice. Expand
Sox 3 Is Required for Gonadal Function , but Not Sex Determination , in Males and Females
Sox3 is expressed in developing gonads and in the brain. Evolutionary evidence suggests that the X-chromosomal Sox3 gene may be the ancestral precursor of Sry, a sex-determining gene, and Sox3 hasExpand
Up-regulation of WNT-4 signaling and dosage-sensitive sex reversal in humans.
TLDR
It is shown that, in testicular Sertoli and Leydig cells, Wnt-4 up-regulates Dax1, a gene known to antagonize the testis-determining factor, Sry, which suggests that mammalian sex determination is sensitive to dosage, at multiple steps in its pathway. Expand
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TLDR
Findings indicate that Ad4BP/SF-1 controls the transcription of the Dax-1 gene, a critical transcription factor in gonadal and adrenal differentiation. Expand
A transcriptional silencing domain in DAX-1 whose mutation causes adrenal hypoplasia congenita.
TLDR
It is shown that the DAX-1 C terminus contains a potent transcriptional silencing activity, which can be transferred to a heterologous DNA-binding domain, which suggests that transcriptionalsilencing by DAx-1 plays a critical role in the pathogenesis of adrenal hypoplasia congenita. Expand
DNA binding and transcriptional repression by DAX-1 blocks steroidogenesis
TLDR
In vitro and in vivo evidence is provided that DAX-1 binds DNA and acts as a powerful transcriptional repressor of StAR gene expression, leading to a drastic decrease in steroid production. Expand
Porcine Dax-1 gene: isolation and expression during gonadal development
TLDR
The isolation of porcine Dax-1 and the analysis of its pattern of expression both during foetal development and in several adult tissues indicates the involvement of DAX-1 in the development and the function of the reproductive system at multiple levels. Expand
Mouse Dax1 expression is consistent with a role in sex determination as well as in adrenal and hypothalamus function
TLDR
The isolation of the mouse Dax1 gene and its pattern of expression during development suggest a basis for adrenal insufficiency and hypogonadotropic hypog onadotropicism in males affected by adrenal hypoplasia congenita and are consistent with a role for DAX1 in gonadal sex determination. Expand
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TLDR
The use of Northern blot analysis and reverse transcription PCR to demonstrate that DAX-1 was expressed in the hypothalamus and the pituitary, and to confirm its expression in adrenal cortex and gonads, indicates the involvement of DAx-1 in the development of the reproductive system at multiple levels within the hypothalamic-pituitary-adrenal/gonadal axis. Expand
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TLDR
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TLDR
Results show that DAX1 is largely, if not solely, responsible for dosage-sensitive sex reversal and provide a model for early events in mammalian sex determination, when precise levels and timing of gene expression are critical. Expand
DAX-1 inhibits SF-1-mediated transactivation via a carboxy-terminal domain that is deleted in adrenal hypoplasia congenita.
TLDR
It is concluded that DAX-1 can inhibit SF-1 transcriptional activity and suggested that the loss of this inhibitory property in DAx-1 may account in part for the phenotype of AHC. Expand
The Wilms’ Tumor Suppressor Gene (wt1) Product Regulates Dax-1 Gene Expression during Gonadal Differentiation
TLDR
The results indicate that the WT1–DAX-1 pathway is an early event in the process of mammalian sex determination, and it is suggested that WT1 regulates Dax-1via GC-rich binding sites found upstream of the DX1 TATA box. Expand
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