TY - JOUR
T1 - Steroidogenic factor 1
T2 - An essential mediator of endocrine development
AU - Parker, Keith L.
AU - Rice, Douglas A.
AU - Lala, Deepak S.
AU - Ikeda, Yayoi
AU - Luo, Xunrong
AU - Wong, Margaret
AU - Bakke, Marit
AU - Zhao, Liping
AU - Frigeri, Claudia
AU - Hanley, Neil A.
AU - Stallings, Nancy
AU - Schimmer, Bernard P.
PY - 2002
Y1 - 2002
N2 - The orphan nuclear receptor steroidogenic factor 1 (SF-1, also called Ad4BP and officially designated NR5A1) has emerged as an essential regulator of endocrine development and function. Initially identified as a tissue-specific transcriptional regulator of the cytochrome P450 steroid hydroxylases, SF-1 has considerably broader roles, as evidenced from studies in knockout mice lacking SF-1. The SF-1-knockout mice lacked adrenal glands and gonads and therefore died from adrenal insufficiency within the first week after birth. In addition, SF-1 knockout mice exhibited male-to-female sex reversal of their internal and external genitalia, impaired expression of multiple markers of pituitary gonadotropes, and agenesis of the ventromedial hypothalamic nucleus (VMH). These studies delineated essential roles of SF-1 in regulating endocrine differentiation and function at multiple levels, particularly with respect to reproduction. This chapter will review the experiments that established SF-1 as a pivotal, global determinant of endocrine differentiation and function. We next discuss recent insights into the mechanisms controlling the expression and function of SF-1 as well as the current status of research aimed at delineating its roles in specific tissues. Finally, we highlight areas where additional studies are needed to expand our understanding of SF-1 action.
AB - The orphan nuclear receptor steroidogenic factor 1 (SF-1, also called Ad4BP and officially designated NR5A1) has emerged as an essential regulator of endocrine development and function. Initially identified as a tissue-specific transcriptional regulator of the cytochrome P450 steroid hydroxylases, SF-1 has considerably broader roles, as evidenced from studies in knockout mice lacking SF-1. The SF-1-knockout mice lacked adrenal glands and gonads and therefore died from adrenal insufficiency within the first week after birth. In addition, SF-1 knockout mice exhibited male-to-female sex reversal of their internal and external genitalia, impaired expression of multiple markers of pituitary gonadotropes, and agenesis of the ventromedial hypothalamic nucleus (VMH). These studies delineated essential roles of SF-1 in regulating endocrine differentiation and function at multiple levels, particularly with respect to reproduction. This chapter will review the experiments that established SF-1 as a pivotal, global determinant of endocrine differentiation and function. We next discuss recent insights into the mechanisms controlling the expression and function of SF-1 as well as the current status of research aimed at delineating its roles in specific tissues. Finally, we highlight areas where additional studies are needed to expand our understanding of SF-1 action.
UR - http://www.scopus.com/inward/record.url?scp=18544386359&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=18544386359&partnerID=8YFLogxK
U2 - 10.1210/rp.57.1.19
DO - 10.1210/rp.57.1.19
M3 - Article
C2 - 12017543
AN - SCOPUS:18544386359
SN - 0079-9963
VL - 57
SP - 19
EP - 36
JO - Recent Progress in Hormone Research
JF - Recent Progress in Hormone Research
ER -