Function of Cyp11a1 in animal models

被引：70

作者：

Hu, MC

Hsu, HJ

Guo, IC

Chung, BC ^{[1
]}

机构：

[1] Acad Sinica, Inst Mol Biol, Taipei 115, Taiwan

[2] Natl Taiwan Univ, Coll Med, Grad Inst Physiol, Taipei 10764, Taiwan

[3] Natl Taiwan Univ, Dept Vet Med, Taipei 10764, Taiwan

来源：

MOLECULAR AND CELLULAR ENDOCRINOLOGY | 2004年 / 215卷 / 1-2期

关键词：

P450scc; SF-1; adrenal; steroidogenesis; steroid; zebrafish; gonad;

D O I：

10.1016/j.mce.2003.11.024

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

CYP11A1 encodes the P450scc enzyme that catalyzes the first and rate-limiting step of steroid biosynthesis. It is expressed in the adrenals and gonads under the control of pituitary peptide hormones. Transcription factor SF-I, which binds to the CYP11A1 promoter, plays an important role in the tissue-specific and hormonally regulated expression. Transgenic mouse experiments linking the CYP11A1 promoter to a reporter gene demonstrate the importance of the SF-1-binding site in directing balanced CYP11A1 gene expression. In addition, targeted mutagenesis of Cypllal also reveals the importance of this gene in steroid secretion, gene regulation, and development of genitalia. Zebrafish cyplal is expressed in the gonads and interrenal glands in the adults, similar to mammals. In addition, it is expressed in yolk syncytial layer during early embryogenesis. These two animal models complement each other for the understanding of gene regulatory pathways in human diseases related to steroid imbalance. (C) 2003 Elsevier Ireland Ltd. All rights reserved.

引用

页码：95 / 100

页数：6

共 39 条

[1] A mutation in the gene encoding steroidogenic factor-1 causes XY sex reversal and adrenal failure in humans [J].