SF-1 expression during adrenal development and tumourigenesis.
SF-1 is a master regulator of steroidogenesis whose expression is critical for normal adrenal and gonadal organogenesis. Strict maintenance of SF-1 levels is essential, and mutations causing under- or overexpression result in congenital adrenal and gonadal defects or hyperplasia, respectively. Data from transgenic mouse models points to a network of transcription factors responsible for stringent regulation of Sf-1 expression during development, which bind to intronic enhancer elements in addition to the basal promoter to specifically modulate transcription in each Sf-1-expressing tissue. Furthermore, analysis of the role of SF-1 in adrenal tumourigenesis implies that improper developmental regulation of Sf-1 expression may have postnatal consequences separate from the well-documented developmental defects.
Division of Cancer Biology, Institute of Cancer Research, London SW36JB, UK.
Jennifer R
JR
Gardiner
Yuichi
Y
Shima
Ken-ichirou
K
Morohashi
Amanda
A
Swain
D000310:Adrenal Gland Neoplasms / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D000311:Adrenal Glands / Q000196:embryology / Q000378:metabolism* / Q000473:pathology; D000818:Animals; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D004742:Enhancer Elements, Genetic; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D006965:Hyperplasia; D007438:Introns; D009363:Neoplasm Proteins / Q000096:biosynthesis / Q000235:genetics; D054339:Steroidogenic Factor 1 / Q000235:genetics / Q000378:metabolism*
D009363:Neoplasm Proteins; D054339:Steroidogenic Factor 1