First Author | Lalli E | Year | 2000 |
Journal | Mol Cell Biol | Volume | 20 |
Issue | 13 | Pages | 4910-21 |
PubMed ID | 10848616 | Mgi Jnum | J:116374 |
Mgi Id | MGI:3694160 | Doi | 10.1128/mcb.20.13.4910-4921.2000 |
Citation | Lalli E, et al. (2000) Orphan receptor DAX-1 is a shuttling RNA binding protein associated with polyribosomes via mRNA. Mol Cell Biol 20(13):4910-21 |
abstractText | The DAX-1 (NR0B1) gene encodes an unusual member of the nuclear hormone receptor superfamily which acts as a transcriptional repressor. Mutations in the human DAX-1 gene cause X-linked adrenal hypoplasia congenita (AHC) associated with hypogonadotropic hypogonadism (HHG). We have studied the intracellular localization of the DAX-1 protein in human adrenal cortex and mouse Leydig tumor cells and found it to be both nuclear and cytoplasmic. A significant proportion of DAX-1 is associated with polyribosomes and is found complexed with polyadenylated RNA. DAX-1 directly binds to RNA, two domains within the protein being responsible for cooperative binding activity and specificity. Mutations in DAX-1 found in AHC-HHG patients significantly impair RNA binding. These findings reveal that DAX-1 plays multiple regulatory roles at the transcriptional and posttranscriptional levels. |