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A cell-specific nuclear receptor is essential for adrenal and gonadal development and sexual differentiation.

Studies in adrenocortical cells have implicated the orphan nuclear receptor SF-1 in the gene regulation of the steroid hydroxylases. We used targeted disruption of the Ftz-F1 gene, which encodes SF-1, to examine its role in intact mice. Despite normal survival in utero, all Ftz-F1 null animals died by postnatal day 8; these animals lacked adrenal glands and gonads and were severely deficient in corticosterone, supporting adrenocortical insufficiency as the probable cause of death. Male and female Ftz-F1 null mice had female internal genitalia, despite complete gonadal agenesis. These studies establish that the Ftz-F1 gene is essential for sexual differentiation and formation of the primary steroidogenic tissues.

Pubmed ID: 8187173


  • Luo X
  • Ikeda Y
  • Parker KL



Publication Data

May 20, 1994

Associated Grants

  • Agency: NHLBI NIH HHS, Id: HL48460

Mesh Terms

  • Adrenal Glands
  • Adrenocorticotropic Hormone
  • Animals
  • Animals, Newborn
  • Apoptosis
  • Base Sequence
  • Cell Movement
  • Cloning, Molecular
  • Corticosterone
  • DNA-Binding Proteins
  • Fallopian Tubes
  • Female
  • Fushi Tarazu Transcription Factors
  • Germ Cells
  • Gonads
  • Homeodomain Proteins
  • Male
  • Mice
  • Mice, Knockout
  • Molecular Sequence Data
  • Receptors, Cytoplasmic and Nuclear
  • Sex Differentiation
  • Steroidogenic Factor 1
  • Transcription Factors