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Gene Review

NR5A2  -  nuclear receptor subfamily 5, group A,...

Homo sapiens

Synonyms: Alpha-1-fetoprotein transcription factor, B1-binding factor, B1F, B1F2, CPF, ...
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Disease relevance of NR5A2


Psychiatry related information on NR5A2

  • The domains included in the FTF were motor skills, executive functions, perception, memory, language, social skills and learning, in addition to a domain for emotional and behavioural problems [4].
  • The FTF and the Child Behavior Checklist (CBCL) were sent to the parents of the children together with questions about background conditions [5].
  • The Five to Fifteen parent questionnaire (FTF) was developed to offer a neuropsychological dimension to the assessment of children with Attention Deficit/Hyperactivity Disorder and other child psychiatric disorders [4].

High impact information on NR5A2

  • The third component is a factor (cleavage and polyadenylation factor, CPF) that is needed for the cleavage reaction and, in addition, confers specificity to the poly(A) polymerase activity; the native molecular weight of CPF is approximately 200 kd [6].
  • Poly(A) polymerase together with CPF is sufficient to specifically polyadenylate pre-mRNA substrates that have been precleaved at the poly(A) addition site [6].
  • One CPF isomer, CPF(DD), preserved CD4-dependent T cell function while inhibiting HIV-1 infection of H9 tumor cells and human T cells [7].
  • In these tissues, LRH-1 plays a predominant role in development, reverse cholesterol transport, bile-acid homeostasis and steroidogenesis [8].
  • LRH-1 is expressed in tissues derived from endoderm, including intestine, liver and exocrine pancreas, as well as in the ovary [8].

Chemical compound and disease context of NR5A2


Biological context of NR5A2


Anatomical context of NR5A2


Associations of NR5A2 with chemical compounds

  • Interestingly, NR5A2 was found to be, by far, the major NR5A subfamily member expressed in the preovulatory follicle and the corpus luteum [11].
  • In the current study we test the hypothesis that liver receptor homologue-1 (LRH; designated NR5A2) is involved in the regulation of steroid hormone production [17].
  • Both SHP proteins specifically interact with the AF-2 transactivation domain of LRH-1 both in vivo and in vitro [12].
  • Interestingly, siRNA-mediated inactivation of LRH-1 decreased the E2-dependent proliferation of MCF7 cells [2].
  • LRH-1 did not augment FSH-stimulated estrogen production, whereas SF-1 produced only a slight ( approximately 30%) augmentation of FSH-stimulated estrogen production [3].

Physical interactions of NR5A2

  • Gel shift assays reveal that Prox1 impairs the binding of hLRH-1 to the promoter of human cyp7a1 gene [18].
  • We present the crystal structure at a resolution of 1.9 A of the ligand-binding domain of hLRH-1 in complex with the NR box 1 motif of human SHP, which we find contacts the AF-2 region of hLRH-1 using selective structural motifs [19].
  • When recombinant MBF2 was added to the HeLa cell nuclear extract in the presence of MBF1 and FTZ622 bearing the DNA-binding region of FTZ-F1, it selectively activated transcription of the fushi tarazu gene [20].

Regulatory relationships of NR5A2

  • Altogether, these data demonstrate that LRH-1 is transcriptionally regulated by the ER alpha and reinforce the hypothesis that LRH-1 could exert potential oncogenic effects during breast cancer formation [2].
  • Dosage-sensitive sex-reversal-adrenal hypoplasia congenita critical region on the X chromosome, gene-1 inhibited LRH-1 stimulated CYP11A1 expression, and that was not overcome by the presence of PKA agonist [21].
  • DAX-1 inhibited LRH-1-stimulated HSD3B2, which was maintained in the presence of dibutyryl cAMP [14].
  • Liver receptor homolog 1 controls the expression of carboxyl ester lipase [13].
  • LRH-1 significantly induced StAR promoter activity in a concentration-dependent manner [22].
  • It was confirmed that suppression in endogenous LRH-1 by small interference RNA stimulates the mRNA levels of certain SREBP target genes and that elevation in active SREBPs in the nucleus in response to cholesterol depletion suppresses the LRH-1 activity [23].
  • We demonstrate that bile acids suppress APOM expression in a SHP-dependent manner in vitro and in vivo by inhibiting LRH-1 transcriptional activity on the APOM promoter as demonstrated by in vivo ChIP assay [24].

Other interactions of NR5A2


Analytical, diagnostic and therapeutic context of NR5A2


  1. Characterization of the genomic structure and tissue-specific promoter of the human nuclear receptor NR5A2 (hB1F) gene. Zhang, C.K., Lin, W., Cai, Y.N., Xu, P.L., Dong, H., Li, M., Kong, Y.Y., Fu, G., Xie, Y.H., Huang, G.M., Wang, Y. Gene (2001) [Pubmed]
  2. The nuclear receptor liver receptor homolog-1 is an estrogen receptor target gene. Annicotte, J.S., Chavey, C., Servant, N., Teyssier, J., Bardin, A., Licznar, A., Badia, E., Pujol, P., Vignon, F., Maudelonde, T., Lazennec, G., Cavailles, V., Fajas, L. Oncogene (2005) [Pubmed]
  3. Liver receptor homolog-1 and steroidogenic factor-1 have similar actions on rat granulosa cell steroidogenesis. Saxena, D., Escamilla-Hernandez, R., Little-Ihrig, L., Zeleznik, A.J. Endocrinology (2007) [Pubmed]
  4. Developmental profiles on the basis of the FTF (Five to Fifteen) questionnaire-clinical validity and utility of the FTF in a child psychiatric sample. Trillingsgaard, A., Damm, D., Sommer, S., Jepsen, J.R., Ostergaard, O., Frydenberg, M., Thomsen, P.H. European child & adolescent psychiatry. (2004) [Pubmed]
  5. Behavioural problems and psychiatric symptoms in 5-13 year-old Swedish children-a comparison of parent ratings on the FTF (Five to Fifteen) with the ratings on CBCL (Child Behavior Checklist). Bohlin, G., Janols, L.O. European child & adolescent psychiatry. (2004) [Pubmed]
  6. 3' cleavage and polyadenylation of mRNA precursors in vitro requires a poly(A) polymerase, a cleavage factor, and a snRNP. Christofori, G., Keller, W. Cell (1988) [Pubmed]
  7. Prevention of HIV-1 infection and preservation of CD4 function by the binding of CPFs to gp120. Finberg, R.W., Diamond, D.C., Mitchell, D.B., Rosenstein, Y., Soman, G., Norman, T.C., Schreiber, S.L., Burakoff, S.J. Science (1990) [Pubmed]
  8. LRH-1: an orphan nuclear receptor involved in development, metabolism and steroidogenesis. Fayard, E., Auwerx, J., Schoonjans, K. Trends Cell Biol. (2004) [Pubmed]
  9. The hepatitis B virus core promoter is strongly activated by the liver nuclear receptor fetoprotein transcription factor or by ectopically expressed steroidogenic factor 1. Gilbert, S., Galarneau, L., Lamontagne, A., Roy, S., Bélanger, L. J. Virol. (2000) [Pubmed]
  10. Functional conservation of interactions between a homeodomain cofactor and a mammalian FTZ-F1 homologue. Steffensen, K.R., Holter, E., Båvner, A., Nilsson, M., Pelto-Huikko, M., Tomarev, S., Treuter, E. EMBO Rep. (2004) [Pubmed]
  11. Expression and regulation of transcripts encoding two members of the NR5A nuclear receptor subfamily of orphan nuclear receptors, steroidogenic factor-1 and NR5A2, in equine ovarian cells during the ovulatory process. Boerboom, D., Pilon, N., Behdjani, R., Silversides, D.W., Sirois, J. Endocrinology (2000) [Pubmed]
  12. Dual mechanisms for repression of the monomeric orphan receptor liver receptor homologous protein-1 by the orphan small heterodimer partner. Lee, Y.K., Moore, D.D. J. Biol. Chem. (2002) [Pubmed]
  13. Liver receptor homolog 1 controls the expression of carboxyl ester lipase. Fayard, E., Schoonjans, K., Annicotte, J.S., Auwerx, J. J. Biol. Chem. (2003) [Pubmed]
  14. The role of the orphan nuclear receptor, liver receptor homologue-1, in the regulation of human corpus luteum 3beta-hydroxysteroid dehydrogenase type II. Peng, N., Kim, J.W., Rainey, W.E., Carr, B.R., Attia, G.R. J. Clin. Endocrinol. Metab. (2003) [Pubmed]
  15. Identification of small molecule agonists of the orphan nuclear receptors liver receptor homolog-1 and steroidogenic factor-1. Whitby, R.J., Dixon, S., Maloney, P.R., Delerive, P., Goodwin, B.J., Parks, D.J., Willson, T.M. J. Med. Chem. (2006) [Pubmed]
  16. Liver receptor homologue-1 is expressed in the adrenal and can regulate transcription of 11 beta-hydroxylase. Wang, Z.N., Bassett, M., Rainey, W.E. J. Mol. Endocrinol. (2001) [Pubmed]
  17. Liver receptor homologue-1 is expressed in human steroidogenic tissues and activates transcription of genes encoding steroidogenic enzymes. Sirianni, R., Seely, J.B., Attia, G., Stocco, D.M., Carr, B.R., Pezzi, V., Rainey, W.E. J. Endocrinol. (2002) [Pubmed]
  18. Prospero-related homeobox (Prox1) is a corepressor of human liver receptor homolog-1 and suppresses the transcription of the cholesterol 7-alpha-hydroxylase gene. Qin, J., Gao, D.M., Jiang, Q.F., Zhou, Q., Kong, Y.Y., Wang, Y., Xie, Y.H. Mol. Endocrinol. (2004) [Pubmed]
  19. Modulation of human nuclear receptor LRH-1 activity by phospholipids and SHP. Ortlund, E.A., Lee, Y., Solomon, I.H., Hager, J.M., Safi, R., Choi, Y., Guan, Z., Tripathy, A., Raetz, C.R., McDonnell, D.P., Moore, D.D., Redinbo, M.R. Nat. Struct. Mol. Biol. (2005) [Pubmed]
  20. Transcriptional activation through interaction of MBF2 with TFIIA. Li, F.Q., Takemaru, K., Goto, M., Ueda, H., Handa, H., Hirose, S. Genes Cells (1997) [Pubmed]
  21. The orphan nuclear receptor, liver receptor homolog-1, regulates cholesterol side-chain cleavage cytochrome p450 enzyme in human granulosa cells. Kim, J.W., Havelock, J.C., Carr, B.R., Attia, G.R. J. Clin. Endocrinol. Metab. (2005) [Pubmed]
  22. Liver receptor homolog-1 regulates the expression of steroidogenic acute regulatory protein in human granulosa cells. Kim, J.W., Peng, N., Rainey, W.E., Carr, B.R., Attia, G.R. J. Clin. Endocrinol. Metab. (2004) [Pubmed]
  23. Interaction between sterol regulatory element-binding proteins and liver receptor homolog-1 reciprocally suppresses their transcriptional activities. Kanayama, T., Arito, M., So, K., Hachimura, S., Inoue, J., Sato, R. J. Biol. Chem. (2007) [Pubmed]
  24. Regulation of anti-atherogenic apolipoprotein M gene expression by the orphan nuclear receptor LRH-1. Venteclef, N., Haroniti, A., Tousaint, J.J., Talianidis, I., Delerive, P. J. Biol. Chem. (2008) [Pubmed]
  25. Transcriptional regulation of the human sterol 12alpha-hydroxylase gene (CYP8B1): roles of heaptocyte nuclear factor 4alpha in mediating bile acid repression. Zhang, M., Chiang, J.Y. J. Biol. Chem. (2001) [Pubmed]
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