title: Steroidogenic factor 1 is the essential transcript of the mouse Ftz-F1 gene.
abstract: Targeted disruption of the mouse Ftz-F1 gene, which encodes the orphan nuclear receptors steroidogenic factor 1 (SF-1) and embryonal long terminal repeat-binding protein (ELP), established that this gene is essential for development of the primary steroidogenic tissues and for male sexual differentiation. Associated with these dramatic developmental abnormalities, all Ftz-F1-disrupted mice died in the immediate postnatal period and had very low glucocorticoid levels. In this report, we show that treatment with corticosteroids markedly prolonged survival of the Ftz-F1-disrupted mice, proving that steroid hormone deficiency causes their death. We also generated SF-1-specific knockout mice with a targeting construct that specifically disrupted the SF-1 coding sequence without impairing the ELP protein. The phenotype of the SF-1-specific knockout mice was indistinguishable from that observed in Ftz-F1-disrupted mice that lack both SF-1 and ELP. Taken together, these results indicate that SF-1 is the Ftz-F1-encoded protein that is required for multiple aspects of endocrine development and for postnatal survival.
mesh_terms: D000305:Adrenal Cortex Hormones / Q000494:pharmacology; D000311:Adrenal Glands / Q000002:abnormalities; D000818:Animals; D004268:DNA-Binding Proteins / Q000737:chemistry / Q000235:genetics* / Q000502:physiology; D005260:Female; D051717:Fushi Tarazu Transcription Factors; D005836:Genitalia, Female / Q000196:embryology; D018398:Homeodomain Proteins; D008297:Male; D008715:Methionine; D051379:Mice; D018345:Mice, Knockout; D016297:Mutagenesis, Site-Directed; D009154:Mutation; D010375:Pedigree; D010641:Phenotype; D018160:Receptors, Cytoplasmic and Nuclear; D012733:Sex Differentiation / Q000235:genetics; D054339:Steroidogenic Factor 1; D013329:Structure-Activity Relationship; D014157:Transcription Factors / Q000737:chemistry / Q000235:genetics* / Q000502:physiology; D014162:Transfection
chemical_list: D000305:Adrenal Cortex Hormones; D004268:DNA-Binding Proteins; D051717:Fushi Tarazu Transcription Factors; D018398:Homeodomain Proteins; D018160:Receptors, Cytoplasmic and Nuclear; D054339:Steroidogenic Factor 1; D014157:Transcription Factors; C515058:steroidogenic factor 1, mouse; D008715:Methionine