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Article

A transcriptional silencing domain in DAX-1 whose mutation causes adrenal hypoplasia congenita.

Molecular endocrinology (Baltimore, Md.) - 1 Dec 1997

Lalli E, Bardoni B, Zazopoulos E, Wurtz J M, Strom T M, Moras D, Sassone-Corsi P

Abstract excerpt

The DAX-1 gene encodes an unusual member of the nuclear hormone receptor superfamily. Mutations in the human DAX-1 gene cause X-linked adrenal hypoplasia congenita associated with hypogonadotropic hypogonadism. We have shown that DAX-1 binds to hairpin secondary structures and blocks steroidogenesis in adrenal cells via transcriptional repression of the steroidogenic acute regulatory protein (StAR) promoter. Here...

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Identifiers

PMID: 9415399
DOI: 10.1210/mend.11.13.0038

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