DAX1

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Name
Dosage-sensitive sex reversal-adrenal hypoplasia congenita critical region on the X chromosome, gene 1
Symbol
DAX1
NRNC Symbol
NR0B1

Overview

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.

Expression

DAX-1 is expressed primarily in reproductive tissues (ovary, testis, uterus), endocrine tissues (adrenal gland) and the central nervous system (CNS) - pituitary and hypothalamus.

View full NURSA DAX-1 expression dataset

Diseases

DAX-1 dysfunction is associated with dosage-sensitive sex reversal, adrenal insufficiency, congenital adrenal hypoplasia with hypogonadotropic hypogonadism.

Phenotypes

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.

View DAX-1 Diseases and Phenotypes section


 Nuclear Receptor Pages User Guide (updated Oct 2008)

At a Glance
Phylogeny
Class
Domain-depleted receptors
NRNC Group
DAX-like receptors (DAX, SHP)

Nomenclature
nuclear receptor subfamily 0, group B, member 1 (NR0B1); adrenal hypoplasia, congenital homolog (human) (AHCH); gonadotropin deficiency (GTD); Dosage-sensitive sex reversal-adrenal hypoplasia congenita critical region on the X chromosome, gene 1 (DAX1)

Primary 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 372, 635-41. View Abstract | View PubMed

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