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Nuclear Receptor Signaling Atlas
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NR0B1
Overview
Synonyms
AHC ; AHCH ; AHX ; Ahc ; Ahch ; DAX ; DAX-1 ; DAX1 ; DSS ; DSS-AHC critical region on the X chromosome protein 1 ; Dax1 ; GTD ; HHG ; NR0B1 ; NROB1 ; Nr0b1 ; SRXY2 ; adrenal hyoplasia protein DAX1 ; adrenal hypoplasia congenita-like protein ; adrenal hypoplasia, congenital homolog ; dax1 ; nr0b1 ; nuclear hormone receptor ; nuclear receptor DAX-1 ; nuclear receptor DAX1 more...
Synonyms
AHC; AHCH; AHX; Ahc; Ahch; DAX; DAX-1; DAX1; DSS; DSS-AHC critical region on the X chromosome protein 1; Dax1; GTD; HHG; NR0B1; NROB1; Nr0b1; SRXY2; adrenal hyoplasia protein DAX1; adrenal hypoplasia congenita-like protein; adrenal hypoplasia, congenital homolog; dax1; nr0b1; nuclear hormone receptor; nuclear receptor DAX-1; nuclear receptor DAX1; nuclear receptor subfamily 0 group B member 1; orphan nuclear receptor DAX-1
NURSA Name
Dosage-sensitive sex reversal-adrenal hypoplasia congenita critical region on the X chromosome, gene 1
Official Symbol
NR0B1
No records found.
Description
The orphan nuclear receptor DAX1 lacks the zinc-finger DNA-binding domain typical of most members of the nuclear receptor (NR) superfamily of transcription factors. It functions by inhibiting the activity of other NRs such as SF-1, ERs and AR by heterodimerization. It is involved in controlling development of the hypothalamic-pituitary axis, as well as in gonadal development and sex determination. DAX-1 is expressed primarily in reproductive tissues (ovary, testis, and uterus), endocrine tissues (adrenal gland) and the central nervous system (CNS) - pituitary and hypothalamus. DAX-1 dysfunction is associated with dosage-sensitive sex reversal, adrenal insufficiency, and congenital adrenal hypoplasia with hypogonadotropic hypogonadism. Targeted disruption (knockout) of the Dax1 gene in mice is associated with failure to maintain spermatogenesis, progressive degeneration of the testicular germinal epithelium and male sterility.
Original References:
Zanaria E, Muscatelli F, Bardoni B, Strom TM, Guioli S, Guo W, Lalli E, Moser C, Walker AP and McCabe ER (1994) An unusual member of the nuclear hormone receptor superfamily responsible for X-linked adrenal hypoplasia congenita. Nature 116 635-41 View Abstract | View PubMed
Ortholog
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