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.
Luo
X
X
Department of Medicine, Duke University Medical Center, Durham, North Carolina 27710.
Ikeda
Y
Y
Parker
K L
KL
D000311:Adrenal Glands / Q000196:embryology*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000818:Animals; D000831:Animals, Newborn; D017209:Apoptosis; D001483:Base Sequence; D002465:Cell Movement; D003001:Cloning, Molecular; D003345:Corticosterone / Q000097:blood; D004268:DNA-Binding Proteins / Q000235:genetics* / Q000502:physiology; D005187:Fallopian Tubes / Q000196:embryology; D005260:Female; D051717:Fushi Tarazu Transcription Factors; D005854:Germ Cells / Q000166:cytology; D006066:Gonads / Q000196:embryology*; D018398:Homeodomain Proteins; D008297:Male; D051379:Mice; D018345:Mice, Knockout / Q000235:genetics; D008969:Molecular Sequence Data; D018160:Receptors, Cytoplasmic and Nuclear / Q000235:genetics* / Q000502:physiology; D012733:Sex Differentiation / Q000235:genetics*; D054339:Steroidogenic Factor 1; D014157:Transcription Factors / Q000235:genetics* / Q000502:physiology
D004268:DNA-Binding Proteins; D051717:Fushi Tarazu Transcription Factors; D018398:Homeodomain Proteins; D018160:Receptors, Cytoplasmic and Nuclear; D054339:Steroidogenic Factor 1; D014157:Transcription Factors; D000324:Adrenocorticotropic Hormone; D003345:Corticosterone