Gene Symbol: Cyp11a1
Description: cytochrome P450, family 11, subfamily a, polypeptide 1
Alias: Cyp11a, Cypxia1, D9Ertd411e, P450scc, Scc, cscc, cholesterol side-chain cleavage enzyme, mitochondrial, cholesterol desmolase, cytochrome P450 11A1, cytochrome P450 side-chain cleavage enzyme, cytochrome P450(scc), cytochrome P450, 11a, cholesterol side-chain cleavage
Species: mouse
Products:     Cyp11a1

Top Publications

  1. Birk O, Casiano D, Wassif C, Cogliati T, Zhao L, Zhao Y, et al. The LIM homeobox gene Lhx9 is essential for mouse gonad formation. Nature. 2000;403:909-13 pubmed
    ..Unlike mice lacking other genes that mediate early stages of gonadogenesis, Lhx9 mutants do not exhibit additional major developmental defects. Thus, LHX9 mutations may underlie certain forms of isolated gonadal agenesis in humans. ..
  2. Jeyasuria P, Ikeda Y, Jamin S, Zhao L, de Rooij D, Themmen A, et al. Cell-specific knockout of steroidogenic factor 1 reveals its essential roles in gonadal function. Mol Endocrinol. 2004;18:1610-9 pubmed
    ..with markedly decreased Leydig cell expression of two essential components of testosterone biosynthesis, Cyp11a and the steroidogenic acute regulatory protein...
  3. Guo I, Huang C, Wang C, Chung B. Activating protein-1 cooperates with steroidogenic factor-1 to regulate 3',5'-cyclic adenosine 5'-monophosphate-dependent human CYP11A1 transcription in vitro and in vivo. Endocrinology. 2007;148:1804-12 pubmed
    The CYP11A1 encodes cytochrome P450scc, catalyzing the first step of steroidogenesis in adrenals and gonads under the control of cAMP-mediated hormonal signals...
  4. Zubair M, Oka S, Parker K, Morohashi K. Transgenic expression of Ad4BP/SF-1 in fetal adrenal progenitor cells leads to ectopic adrenal formation. Mol Endocrinol. 2009;23:1657-67 pubmed publisher
    ..The effects of FAdE-controlled Ad4BP/SF-1 overexpression in mice provide a novel model of ectopic adrenal formation that further supports the critical role of Ad4BP/SF-1 in the determination of steroidogenic cell fate in vivo. ..
  5. Hu M, Hsu N, El Hadj N, Pai C, Chu H, Wang C, et al. Steroid deficiency syndromes in mice with targeted disruption of Cyp11a1. Mol Endocrinol. 2002;16:1943-50 pubmed
    ..deficiency in more detail, we used a gene-targeting technique to insert a neo gene into the first exon to disrupt Cyp11a1, the first gene in steroid biosynthetic pathways. Cyp11a1 null mice do not synthesize steroids...
  6. Cole T, Blendy J, Monaghan A, Krieglstein K, Schmid W, Aguzzi A, et al. Targeted disruption of the glucocorticoid receptor gene blocks adrenergic chromaffin cell development and severely retards lung maturation. Genes Dev. 1995;9:1608-21 pubmed
  7. Jeays Ward K, Hoyle C, Brennan J, Dandonneau M, Alldus G, Capel B, et al. Endothelial and steroidogenic cell migration are regulated by WNT4 in the developing mammalian gonad. Development. 2003;130:3663-70 pubmed
    ..These studies provide a model for understanding how the same signalling molecule can act on two different cell types to coordinate sex development. ..
  8. King P, Paul A, Laufer E. Shh signaling regulates adrenocortical development and identifies progenitors of steroidogenic lineages. Proc Natl Acad Sci U S A. 2009;106:21185-90 pubmed publisher
    ..Our findings both support a novel dual lineage, Shh-independent and Shh-dependent, model of adrenocortical development, and identify distinct populations of adrenocortical progenitor and candidate stem cells...
  9. Polanco J, Wilhelm D, Davidson T, Knight D, Koopman P. Sox10 gain-of-function causes XX sex reversal in mice: implications for human 22q-linked disorders of sex development. Hum Mol Genet. 2010;19:506-16 pubmed publisher
    ..1, our results functionally implicate SOX10 in the etiology of these DSDs. ..

More Information


  1. Ikeda Y, Shen W, Ingraham H, Parker K. Developmental expression of mouse steroidogenic factor-1, an essential regulator of the steroid hydroxylases. Mol Endocrinol. 1994;8:654-62 pubmed
    ..Consistent with its proposed role in regulating cholesterol side-chain cleavage enzyme (SCC), SF-1 is expressed before SCC...
  2. Val P, Jeays Ward K, Swain A. Identification of a novel population of adrenal-like cells in the mammalian testis. Dev Biol. 2006;299:250-6 pubmed
    ..Consistent with this, using transgenic Cyp11a1 reporter mice, we definitively show that steroidogenic cells can migrate from the mesonephros into the XY gonad...
  3. Buaas F, Gardiner J, Clayton S, Val P, Swain A. In vivo evidence for the crucial role of SF1 in steroid-producing cells of the testis, ovary and adrenal gland. Development. 2012;139:4561-70 pubmed publisher
    ..This study is the first to show the in vivo requirements of SF1 in steroidogenesis and provides novel data on the cellular consequences of the loss of this protein specifically within steroid-producing cells. ..
  4. Chassot A, Ranc F, Gregoire E, Roepers Gajadien H, Taketo M, Camerino G, et al. Activation of beta-catenin signaling by Rspo1 controls differentiation of the mammalian ovary. Hum Mol Genet. 2008;17:1264-77 pubmed publisher
    ..Thus, a balance between Sox9 and beta-catenin activation determines the fate of the gonad, with Rspo1 acting as a crucial regulator of canonical beta-catenin signaling required for female development. ..
  5. Kim A, Reuter A, Zubair M, Else T, Serecky K, Bingham N, et al. Targeted disruption of beta-catenin in Sf1-expressing cells impairs development and maintenance of the adrenal cortex. Development. 2008;135:2593-602 pubmed publisher
    ..These results define crucial roles for beta-catenin--presumably as part of the Wnt canonical signaling pathway--in both embryonic development of the adrenal cortex and in maintenance of the adult organ. ..
  6. Chung B. Steroid deficiency syndromes in mice with targeted disruption of Cyp11a1. Endocr Res. 2002;28:575 pubmed
  7. Jeays Ward K, Dandonneau M, Swain A. Wnt4 is required for proper male as well as female sexual development. Dev Biol. 2004;276:431-40 pubmed
    ..These results identify WNT4 as a new factor involved in the mammalian testis determination pathway and show that genes can have a specific but distinct role in both male and female gonad development. ..
  8. Yao H, Whoriskey W, Capel B. Desert Hedgehog/Patched 1 signaling specifies fetal Leydig cell fate in testis organogenesis. Genes Dev. 2002;16:1433-40 pubmed
  9. Barrionuevo F, Georg I, Scherthan H, Lecureuil C, Guillou F, Wegner M, et al. Testis cord differentiation after the sex determination stage is independent of Sox9 but fails in the combined absence of Sox9 and Sox8. Dev Biol. 2009;327:301-12 pubmed publisher
    ..This study shows that testis cord differentiation is independent of Sox9, and that concerted Sox9 and Sox8 function in post E14.0 Sertoli cells is essential for the maintenance of testicular function. ..
  10. Lavery R, Lardenois A, Ranc Jianmotamedi F, Pauper E, Gregoire E, Vigier C, et al. XY Sox9 embryonic loss-of-function mouse mutants show complete sex reversal and produce partially fertile XY oocytes. Dev Biol. 2011;354:111-22 pubmed publisher
    ..Taken together, we found that XY Sf1:Cre(Tg/+); Sox9(flox/flox) females are capable of producing viable offspring albeit at a reduced level. ..
  11. Lavery R, Chassot A, Pauper E, Gregoire E, Klopfenstein M, de Rooij D, et al. Testicular differentiation occurs in absence of R-spondin1 and Sox9 in mouse sex reversals. PLoS Genet. 2012;8:e1003170 pubmed publisher
    ..The dogma that considers female differentiation as a default pathway therefore needs to be definitively revised. ..
  12. Miyagawa S, Matsumaru D, Murashima A, Omori A, Satoh Y, Haraguchi R, et al. The role of sonic hedgehog-Gli2 pathway in the masculinization of external genitalia. Endocrinology. 2011;152:2894-903 pubmed publisher
    ..The current study identified hedgehog signaling pathway as a key factor not only for initial development but also for sexually dimorphic development of the external genitalia in coordination with androgen signaling. ..
  13. Callier P, Calvel P, Matevossian A, Makrythanasis P, Bernard P, Kurosaka H, et al. Loss of function mutation in the palmitoyl-transferase HHAT leads to syndromic 46,XY disorder of sex development by impeding Hedgehog protein palmitoylation and signaling. PLoS Genet. 2014;10:e1004340 pubmed publisher
    ..Furthermore, they provide the first clinical evidence of the essential role played by lipid modification of Hh proteins in human testicular organogenesis and embryonic development. ..
  14. Nef S, Verma Kurvari S, Merenmies J, Vassalli J, Efstratiadis A, Accili D, et al. Testis determination requires insulin receptor family function in mice. Nature. 2003;426:291-5 pubmed
    ..Reduced expression of both Sry and the early testis-specific marker Sox9 indicates that the insulin signalling pathway is required for male sex determination. ..
  15. Park S, Meeks J, Raverot G, Pfaff L, Weiss J, Hammer G, et al. Nuclear receptors Sf1 and Dax1 function cooperatively to mediate somatic cell differentiation during testis development. Development. 2005;132:2415-23 pubmed
    ..In studies of the developing testis, expression of the fetal Leydig cell markers Cyp17 and Cyp11a1 was reduced in heterozygous Sf1-deficient mice at E13.5, consistent with dose-dependent effects of Sf1...
  16. Lan H, Wu C, Shih H, Chung B. Death-associated protein 6 (Daxx) mediates cAMP-dependent stimulation of Cyp11a1 (P450scc) transcription. J Biol Chem. 2012;287:5910-6 pubmed publisher
    ..SF-1 is a key transcription factor for all steroidogenic genes. It up-regulates the expression of the steroidogenic Cyp11a1 gene in the adrenal in a pathway stimulated by cAMP through HIPK3-mediated JNK/c-Jun phosphorylation...
  17. Chawengsaksophak K, Svingen T, Ng E, Epp T, Spiller C, Clark C, et al. Loss of Wnt5a disrupts primordial germ cell migration and male sexual development in mice. Biol Reprod. 2012;86:1-12 pubmed publisher
    ..Our results indicate multiple roles for Wnt5a during mammalian reproductive development and help to clarify further the etiology of Robinow syndrome (OMIM 268310), a disease previously linked to the WNT5A pathway. ..
  18. Schnabel C, Selleri L, Cleary M. Pbx1 is essential for adrenal development and urogenital differentiation. Genesis. 2003;37:123-30 pubmed
    ..These findings establish a Pbx1-dependent pathway that regulates the expansion of SF-1 positive cells essential for adrenal formation and gonadal differentiation and demonstrate an early requirement for Pbx1 in urogenital development. ..
  19. Koizumi M, Oyama K, Yamakami Y, Kida T, Satoh R, Kato S, et al. Lgr4 controls specialization of female gonads in mice. Biol Reprod. 2015;93:90 pubmed publisher
    ..This study suggests that Lgr4 is critical for ovarian somatic cell specialization via the cooperative signaling of Rspo1 and Wnt/beta-catenin. ..
  20. Chiu S, Asai N, Costantini F, Hsu W. SUMO-specific protease 2 is essential for modulating p53-Mdm2 in development of trophoblast stem cell niches and lineages. PLoS Biol. 2008;6:e310 pubmed publisher
    ..Our data reveal a key genetic pathway, SENP2-Mdm2-p53, underlying trophoblast lineage development, suggesting its pivotal role in cell cycle progression of mitosis and endoreduplication. ..
  21. Hsu N, Shih M, Chung B. Study of the function of proximal SF-1 binding sites on Cyp11A1 promoter. Endocr Res. 2004;30:813-4 pubmed
  22. Morohashi K, Tsuboi Asai H, Matsushita S, Suda M, Nakashima M, Sasano H, et al. Structural and functional abnormalities in the spleen of an mFtz-F1 gene-disrupted mouse. Blood. 1999;93:1586-94 pubmed
    ..Our results indicate that a lack of the mFtz-F1 gene in mice is associated with structural and functional abnormalities of the splenic vascular system. ..
  23. Val P, Martinez Barbera J, Swain A. Adrenal development is initiated by Cited2 and Wt1 through modulation of Sf-1 dosage. Development. 2007;134:2349-58 pubmed
    ..These results highlight the importance of transcription factor dosage in organogenesis and the role of transcription co-factors such as Cited2 in determining the levels of these factors. ..
  24. Nishida H, Miyagawa S, Vieux Rochas M, Morini M, Ogino Y, Suzuki K, et al. Positive regulation of steroidogenic acute regulatory protein gene expression through the interaction between Dlx and GATA-4 for testicular steroidogenesis. Endocrinology. 2008;149:2090-7 pubmed publisher
    ..These results suggest that Dlx5 and Dlx6 participate in the control of steroidogenesis during testis development. The findings of this study may open the way to analyze human congenital birth defects. ..
  25. Paul A, Laufer E. Endogenous biotin as a marker of adrenocortical cells with steroidogenic potential. Mol Cell Endocrinol. 2011;336:133-40 pubmed publisher
    ..Fluorescent streptavidin applied using a simple one-step staining protocol thus provides a potent counterstain for use in adrenal analyses. ..
  26. Huang C, Shih M, Hsu N, Chien Y, Chung B. Fetal glucocorticoid synthesis is required for development of fetal adrenal medulla and hypothalamus feedback suppression. Endocrinology. 2012;153:4749-56 pubmed
    ..We have created mice with a disruption of the Cyp11a1 gene resulting in loss of fetal steroid secretion but preserving the maternal supply...
  27. Colvin J, Green R, Schmahl J, Capel B, Ornitz D. Male-to-female sex reversal in mice lacking fibroblast growth factor 9. Cell. 2001;104:875-89 pubmed
    ..While Sry is found only in some mammals, Fgfs are highly conserved. Thus, Fgfs may function in sex determination and reproductive system development in many species. ..
  28. Barsoum I, Yao H. Redundant and differential roles of transcription factors Gli1 and Gli2 in the development of mouse fetal Leydig cells. Biol Reprod. 2011;84:894-9 pubmed publisher
    ..Our results reveal overlapping and redundant Gli1 and Gli2 roles in fetal Leydig cell differentiation and a novel regulation of Gli2 expression in the fetal testis...
  29. Stromstedt M, Keeney D, Waterman M, Paria B, Conley A, Dey S. Preimplantation mouse blastocysts fail to express CYP genes required for estrogen biosynthesis. Mol Reprod Dev. 1996;43:428-36 pubmed
    ..lanosterol 14-demethylase P450 (CYP51), 17 alpha-hydroxylase P450 (CYP17), cholesterol side-chain cleavage P450 (CYP11A1), sterol 27-hydroxylase P450 (CYP27), and aromatase P450 (CYP19) were examined in day 4 mouse blastocysts (day 1 =..
  30. Wilhelm D, Washburn L, Truong V, Fellous M, Eicher E, Koopman P. Antagonism of the testis- and ovary-determining pathways during ovotestis development in mice. Mech Dev. 2009;126:324-36 pubmed publisher
    ..Together these findings provide important new insights into the molecular regulation of testis and ovary development. ..
  31. Liljander M, Andersson A, Holmdahl R, Mattsson R. Increased litter size and super-ovulation rate in congenic C57BL mice carrying a polymorphic fragment of NFR/N origin at the Fecq4 locus of chromosome 9. Genet Res (Camb). 2009;91:259-65 pubmed publisher
    ..Genes of the cytochrome P450 family (1, 11 and 19), such as Cyp19a1, are assumed to be particularly interesting, since they are known to exhibit female-associated reproductive phenotypes, affecting the ovulation rate, if mutated. ..
  32. Zhang L, Chen M, Wen Q, Li Y, Wang Y, Wang Y, et al. Reprogramming of Sertoli cells to fetal-like Leydig cells by Wt1 ablation. Proc Natl Acad Sci U S A. 2015;112:4003-8 pubmed publisher
    ..This study thus provides a novel concept for somatic cell fate determination in testis development that may also represent an etiology of male infertility in human patients. ..
  33. Combes A, Bowles J, Feng C, Chiu H, Khoo P, Jackson A, et al. Expression and functional analysis of Dkk1 during early gonadal development. Sex Dev. 2011;5:124-30 pubmed publisher
    ..Nor did we find significant differences in expression of key markers of testis and ovarian development. We propose that DKK1 may play a protective role that is not unmasked by loss-of-function in the absence of other stressors. ..
  34. Pihlajoki M, Gretzinger E, Cochran R, Kyrönlahti A, Schrade A, Hiller T, et al. Conditional mutagenesis of Gata6 in SF1-positive cells causes gonadal-like differentiation in the adrenal cortex of mice. Endocrinology. 2013;154:1754-67 pubmed publisher
    ..Our findings offer genetic proof that GATA6 regulates the differentiation of steroidogenic progenitors into adrenocortical cells. ..
  35. Notini A, McClive P, Meachem S, van den Bergen J, Western P, Gustin S, et al. Redd1 is a novel marker of testis development but is not required for normal male reproduction. Sex Dev. 2012;6:223-30 pubmed publisher
    ..Our results show that Redd1 alone is not required for testis development or fertility in mice. The lack of a male reproductive phenotype in Redd1 mice may be due to functional compensation by the related gene Redd2. ..
  36. Hammer F, Compagnone N, Vigne J, Bair S, Mellon S. Transcriptional regulation of P450scc gene expression in the embryonic rodent nervous system. Endocrinology. 2004;145:901-12 pubmed
    ..with the conversion of cholesterol to pregnenolone, catalyzed by mitochondrial P450 side-chain cleavage enzyme (P450scc)...
  37. Tang H, Brennan J, Karl J, Hamada Y, Raetzman L, Capel B. Notch signaling maintains Leydig progenitor cells in the mouse testis. Development. 2008;135:3745-53 pubmed publisher
  38. Compagnone N, Bulfone A, Rubenstein J, Mellon S. Expression of the steroidogenic enzyme P450scc in the central and peripheral nervous systems during rodent embryogenesis. Endocrinology. 1995;136:2689-96 pubmed
    ..We previously demonstrated that the messenger RNAs for the cholesterol side-chain cleavage enzyme, P450scc, and one form of 11 beta-hydroxylase, P450c11 beta, are regionally expressed in the adult rat brain...
  39. Kyrönlahti A, Vetter M, Euler R, Bielinska M, Jay P, Anttonen M, et al. GATA4 deficiency impairs ovarian function in adult mice. Biol Reprod. 2011;84:1033-44 pubmed publisher
    ..released fewer oocytes, produced less estrogen, and expressed less mRNA for the putative GATA4 target genes Star, Cyp11a1, and Cyp19...
  40. Chassot A, Bradford S, Auguste A, Gregoire E, Pailhoux E, de Rooij D, et al. WNT4 and RSPO1 together are required for cell proliferation in the early mouse gonad. Development. 2012;139:4461-72 pubmed publisher
    ..Hence, this study identifies Rspo1 and Wnt4 as two new regulators of cell proliferation in the early gonad regardless of its sex, in addition to the specific role of these genes in ovarian differentiation. ..
  41. Chadwick J, Swager S, Lowe J, Welc S, Tidball J, Gomez Sanchez C, et al. Myeloid cells are capable of synthesizing aldosterone to exacerbate damage in muscular dystrophy. Hum Mol Genet. 2016;25:5167-5177 pubmed publisher
    ..This first report of myeloid cells having the capacity to produce aldosterone may have implications for a wide variety of acute injuries and chronic diseases with inflammation where MR antagonists may be therapeutic. ..
  42. Sher N, Yivgi Ohana N, Orly J. Transcriptional regulation of the cholesterol side chain cleavage cytochrome P450 gene (CYP11A1) revisited: binding of GATA, cyclic adenosine 3',5'-monophosphate response element-binding protein and activating protein (AP)-1 proteins to a distal nove. Mol Endocrinol. 2007;21:948-62 pubmed
    ..and key enzyme controlling the synthesis of steroid hormones is cholesterol side chain cleavage cytochrome P450 (P450scc, CYP11A1)...
  43. Bowles J, Feng C, Knight D, Smith C, Roeszler K, Bagheri Fam S, et al. Male-specific expression of Aldh1a1 in mouse and chicken fetal testes: implications for retinoid balance in gonad development. Dev Dyn. 2009;238:2073-80 pubmed publisher
    ..Our data suggest that low levels of RA may be required for early developmental events in the testis, or that Aldh1a1 expression in the fetus may prefigure a later requirement for ALDH1A1 in regulating spermatogenesis postnatally. ..
  44. Zhang J, Yuan Y, Liu Q, Yang D, Liu M, Shen L, et al. Differentially expressed genes in the testicular tissues of adenylyl cyclase 3 knockout mice. Gene. 2017;602:33-42 pubmed publisher
    ..These data would help elucidate the general understanding of the mechanisms underlying the sterility in AC3-/- male mice. ..
  45. Suto J, Sekikawa K. Quantitative trait locus analysis of plasma cholesterol and triglyceride levels in KK x RR F2 mice. Biochem Genet. 2003;41:325-41 pubmed
    ..Identification of distinct, but related traits in an identical chromosomal region will facilitate revealing the responsible gene. ..
  46. Rice D, Kirkman M, Aitken L, Mouw A, Schimmer B, Parker K. Analysis of the promoter region of the gene encoding mouse cholesterol side-chain cleavage enzyme. J Biol Chem. 1990;265:11713-20 pubmed
    The cholesterol side-chain cleavage enzyme (SCC) catalyzes the initial and rate-limiting step in the synthesis of steroid hormones. The mouse gene encoding SCC was cloned and the nucleotide sequence of its 5'-flanking region determined...
  47. Keeney D, Ikeda Y, Waterman M, Parker K. Cholesterol side-chain cleavage cytochrome P450 gene expression in the primitive gut of the mouse embryo does not require steroidogenic factor 1. Mol Endocrinol. 1995;9:1091-8 pubmed
    In situ hybridization studies reveal novel sites of expression of cholesterol side-chain cleavage cytochrome P450 (P450scc) during murine embryonic development...
  48. Ran H, Kong S, Zhang S, Cheng J, Zhou C, He B, et al. Nuclear Shp2 directs normal embryo implantation via facilitating the ERα tyrosine phosphorylation by the Src kinase. Proc Natl Acad Sci U S A. 2017;114:4816-4821 pubmed publisher
    ..Besides uncovering a regulatory mechanism, this study could be clinically relevant to dysfunctional ERα-caused endometrial disorders in women. ..
  49. Keeney D. Perspectives in steroid hydroxylase gene expression: novel sites of expression during embryonic development. Endocr Res. 1995;21:103-7 pubmed
    ..b>CYP11A is expressed in the fetal adrenal but also in developing hindgut, which will form the colon, and in cells located ..
  50. Minenko A, Novikova L, Luzikov V, Kovaleva I. Import of hybrid forms of CYP11A1 into yeast mitochondria. Biochim Biophys Acta. 2008;1780:1121-30 pubmed publisher
    ..To ensure insertion of the CYP11A1 domain into the inner mitochondrial membrane during the process of translocation, topogenic sequences containing ..
  51. Rakoczy J, Fernandez Valverde S, Glazov E, Wainwright E, Sato T, Takada S, et al. MicroRNAs-140-5p/140-3p modulate Leydig cell numbers in the developing mouse testis. Biol Reprod. 2013;88:143 pubmed publisher
  52. Vanhoutteghem A, Messiaen S, Hervé F, Delhomme B, Moison D, Petit J, et al. The zinc-finger protein basonuclin 2 is required for proper mitotic arrest, prevention of premature meiotic initiation and meiotic progression in mouse male germ cells. Development. 2014;141:4298-310 pubmed publisher
    ..In view of the extreme evolutionary conservation of BNC2, the findings described here are likely to apply to many species. ..
  53. Wong M, Rice D, Parker K, Schimmer B. The roles of cAMP and cAMP-dependent protein kinase in the expression of cholesterol side chain cleavage and steroid 11 beta-hydroxylase genes in mouse adrenocortical tumor cells. J Biol Chem. 1989;264:12867-71 pubmed
    The expression of the genes encoding cholesterol side chain cleavage enzyme (SCC) and steroid 11 beta-hydroxylase (11 beta-OHase) was examined in Y1 mouse adrenocortical tumor cells and in derivative cell lines defective in cAMP-..
  54. Chien Y, Cheng W, Wu M, Jiang S, Shen C, Chung B. Misregulated progesterone secretion and impaired pregnancy in Cyp11a1 transgenic mice. Biol Reprod. 2013;89:91 pubmed publisher
    ..levels of progesterone (P4), which is secreted from ovarian luteal cells via enzymatic steps catalyzed by P450scc (CYP11A1) and HSD3B...
  55. O Hara L, York J, Zhang P, Smith L. Targeting of GFP-Cre to the mouse Cyp11a1 locus both drives cre recombinase expression in steroidogenic cells and permits generation of Cyp11a1 knock out mice. PLoS ONE. 2014;9:e84541 pubmed publisher
    ..a transgenic mouse line that expresses Cre Recombinase under the regulation of the endogenous Cytochrome P450 side chain cleavage enzyme (Cyp11a1) promoter...
  56. Wainwright E, Svingen T, Ng E, Wicking C, Koopman P. Primary cilia function regulates the length of the embryonic trunk axis and urogenital field in mice. Dev Biol. 2014;395:342-54 pubmed publisher
  57. Keeney D, Jenkins C, Waterman M. Developmentally regulated expression of adrenal 17 alpha-hydroxylase cytochrome P450 in the mouse embryo. Endocrinology. 1995;136:4872-9 pubmed
  58. Schmahl J, Kim Y, Colvin J, Ornitz D, Capel B. Fgf9 induces proliferation and nuclear localization of FGFR2 in Sertoli precursors during male sex determination. Development. 2004;131:3627-36 pubmed
    ..The nuclear localization of FGFR2 coincides with the initiation of Sry expression and the nuclear localization of SOX9 during the early differentiation of Sertoli cells and the determination of male fate. ..
  59. Shawki H, Oishi H, Usui T, Kitadate Y, Basha W, Abdellatif A, et al. MAFB is dispensable for the fetal testis morphogenesis and the maintenance of spermatogenesis in adult mice. PLoS ONE. 2018;13:e0190800 pubmed publisher
  60. Mellon S, Deschepper C. Neurosteroid biosynthesis: genes for adrenal steroidogenic enzymes are expressed in the brain. Brain Res. 1993;629:283-92 pubmed
    ..We detected mRNAs for adrenodoxin, P450scc (cholesterol side-chain cleavage enzyme) and P450c11 beta (11 beta-hydroxylase) but not for P450c17 (17 alpha-..
  61. Dumbell R, Leliavski A, Matveeva O, Blaum C, Tsang A, Oster H. Dissociation of Molecular and Endocrine Circadian Rhythms in Male Mice Lacking Bmal1 in the Adrenal Cortex. Endocrinology. 2016;157:4222-4233 pubmed
  62. Liu C, Peng J, Matzuk M, Yao H. Lineage specification of ovarian theca cells requires multicellular interactions via oocyte and granulosa cells. Nat Commun. 2015;6:6934 pubmed publisher
    ..Our studies provide the first genetic evidence for the origins of theca cells and reveal a multicellular interaction critical for the formation of a functional theca. ..
  63. Tevosian S, Albrecht K, Crispino J, Fujiwara Y, Eicher E, Orkin S. Gonadal differentiation, sex determination and normal Sry expression in mice require direct interaction between transcription partners GATA4 and FOG2. Development. 2002;129:4627-34 pubmed
    ..crucial for normal Sertoli cell function (Sox9, Mis and Dhh) and three Leydig cell steroid biosynthetic enzymes (p450scc, 3betaHSD and p450c17) were not expressed in XY Fog2(-/-) and Gata(ki/ki) gonads, whereas Wnt4, a gene required ..
  64. Tomaszewski J, Joseph A, Archambeault D, Yao H. Essential roles of inhibin beta A in mouse epididymal coiling. Proc Natl Acad Sci U S A. 2007;104:11322-7 pubmed
    ..These results demonstrated that Inhba, a mesenchyme-specific gene, acts collectively with testosterone to facilitate epididymal coiling by stimulating epithelial proliferation. ..
  65. Huang C, Kraft C, Moy N, Ng L, Forrest D. A Novel Population of Inner Cortical Cells in the Adrenal Gland That Displays Sexually Dimorphic Expression of Thyroid Hormone Receptor-β1. Endocrinology. 2015;156:2338-48 pubmed publisher
    ..TRβ1-deficient mice were resistant to these actions of T3, supporting a functional role for TRβ1 in the inner cortex. ..
  66. Yao H, Matzuk M, Jorgez C, Menke D, Page D, Swain A, et al. Follistatin operates downstream of Wnt4 in mammalian ovary organogenesis. Dev Dyn. 2004;230:210-5 pubmed
    ..Thus, we propose that WNT4 acts through FST to regulate vascular boundaries and maintain germ cell survival in the ovary. ..
  67. Cui S, Ross A, Stallings N, Parker K, Capel B, Quaggin S. Disrupted gonadogenesis and male-to-female sex reversal in Pod1 knockout mice. Development. 2004;131:4095-105 pubmed
    ..Within Pod1 KO gonads, the number of cells expressing the cholesterol side-chain cleavage enzyme (Scc) was increased markedly...
  68. Miyado M, Nakamura M, Miyado K, Morohashi K, Sano S, Nagata E, et al. Mamld1 deficiency significantly reduces mRNA expression levels of multiple genes expressed in mouse fetal Leydig cells but permits normal genital and reproductive development. Endocrinology. 2012;153:6033-40 pubmed publisher
    ..but significantly reduced testicular mRNA levels (65-80%) of genes exclusively expressed in Leydig cells (Star, Cyp11a1, Cyp17a1, Hsd3b1, and Insl3) as well as grossly normal testicular mRNA levels of genes expressed in other cell ..
  69. Elumalai P, Krishnamoorthy G, Selvakumar K, Arunkumar R, Venkataraman P, Arunakaran J. Studies on the protective role of lycopene against polychlorinated biphenyls (Aroclor 1254)-induced changes in StAR protein and cytochrome P450 scc enzyme expression on Leydig cells of adult rats. Reprod Toxicol. 2009;27:41-5 pubmed publisher
    ..Testes were removed and Leydig cells were isolated in aseptic condition. StAR protein, cytochrome P450 scc enzyme expression were studied by Western blot analysis and 3beta-HSD activity was estimated ..
  70. Guasti L, Candy Sze W, McKay T, Grose R, King P. FGF signalling through Fgfr2 isoform IIIb regulates adrenal cortex development. Mol Cell Endocrinol. 2013;371:182-8 pubmed publisher
    ..Fgfr2 ligands were detected in both the capsule and the cortex, suggesting the importance of signalling between the capsule and the cortex in adrenal development. ..
  71. Ikeda Y, Tanaka H, Esaki M. Effects of gestational diethylstilbestrol treatment on male and female gonads during early embryonic development. Endocrinology. 2008;149:3970-9 pubmed publisher
    ..5. The results suggest that exogenous estrogens can alter sex-specific genetic pathways governing early differentiation and cell proliferation of both male and female gonads. ..
  72. Tohonen V, Frygelius J, Mohammadieh M, Kvist U, Pelliniemi L, O BRIEN K, et al. Normal sexual development and fertility in testatin knockout mice. Mol Cell Biol. 2005;25:4892-902 pubmed
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