Gene Symbol: Pax2
Description: paired box 2
Alias: Pax-2, paired box protein Pax-2, paired box gene 2, paired box homeotic gene 2, paired box protein 2
Species: rat
Products:     Pax2

Top Publications

  1. Zou D, Silvius D, Rodrigo Blomqvist S, Enerback S, Xu P. Eya1 regulates the growth of otic epithelium and interacts with Pax2 during the development of all sensory areas in the inner ear. Dev Biol. 2006;298:430-41 pubmed
    ..they interact with Eya1 or Six1 during inner ear morphogenesis, we have analyzed the expression pattern of Eya1, Pax2 and Pax8 on adjacent sections of otic epithelium from E8.5 to 9...
  2. Grote D, Souabni A, Busslinger M, Bouchard M. Pax 2/8-regulated Gata 3 expression is necessary for morphogenesis and guidance of the nephric duct in the developing kidney. Development. 2006;133:53-61 pubmed
    ..The transcription factors Pax2 and Pax8 are essential for the initiation of pro- and mesonephros development...
  3. Zhang S, Moini B, Ingelfinger J. Angiotensin II increases Pax-2 expression in fetal kidney cells via the AT2 receptor. J Am Soc Nephrol. 2004;15:1452-65 pubmed
  4. Gong K, Yallowitz A, Sun H, Dressler G, Wellik D. A Hox-Eya-Pax complex regulates early kidney developmental gene expression. Mol Cell Biol. 2007;27:7661-8 pubmed
    ..Herein, we demonstrate that Hox11 paralogous proteins form a complex with Pax2 and Eya1 to directly activate expression of Six2 and Gdnf in the metanephric mesenchyme...
  5. Burton Q, Cole L, Mulheisen M, Chang W, Wu D. The role of Pax2 in mouse inner ear development. Dev Biol. 2004;272:161-75 pubmed
    The paired box transcription factor, Pax2, is important for cochlear development in the mouse inner ear...
  6. Torres M, Gómez Pardo E, Gruss P. Pax2 contributes to inner ear patterning and optic nerve trajectory. Development. 1996;122:3381-91 pubmed
    During gestation, the paired box-containing gene Pax2 is expressed in the mid-hindbrain area, developing eye and inner ear...
  7. Maeshima A, Maeshima K, Nojima Y, Kojima I. Involvement of Pax-2 in the action of activin A on tubular cell regeneration. J Am Soc Nephrol. 2002;13:2850-9 pubmed
    ..These results suggest that activin A regulates tubular cell growth and differentiation by modulating the expression of Pax-2 during regeneration. ..
  8. Huang B, Pi L, Chen C, Yuan F, Zhou Q, Teng J, et al. WT1 and Pax2 re-expression is required for epithelial-mesenchymal transition in 5/6 nephrectomized rats and cultured kidney tubular epithelial cells. Cells Tissues Organs. 2012;195:296-312 pubmed publisher
    ..The Wilms' tumor suppressor gene WT1 and the paired box 2 gene Pax2 are needed to induce mesenchymal-epithelial transition and play key roles in the progression of nephrogenesis...
  9. Bouchard M, Souabni A, Mandler M, Neubüser A, Busslinger M. Nephric lineage specification by Pax2 and Pax8. Genes Dev. 2002;16:2958-70 pubmed
    ..nephric lineage is specified during pronephros induction, no single regulator, including the transcription factor Pax2 or Pax8, has yet been identified to control this initial phase of kidney development...

More Information


  1. Narlis M, Grote D, Gaitan Y, Boualia S, Bouchard M. Pax2 and pax8 regulate branching morphogenesis and nephron differentiation in the developing kidney. J Am Soc Nephrol. 2007;18:1121-9 pubmed
    Pax genes are important regulators of kidney development. In the mouse, homozygous Pax2 inactivation results in renal agenesis, a phenotype that has largely precluded the analysis of Pax gene function during metanephric kidney development...
  2. Murawski I, Myburgh D, Favor J, Gupta I. Vesico-ureteric reflux and urinary tract development in the Pax2 1Neu+/- mouse. Am J Physiol Renal Physiol. 2007;293:F1736-45 pubmed
    ..roughly one-third of patients with renal-coloboma syndrome, an autosomal dominant condition caused by a mutation in PAX2. Here, we report that a mouse model with an identical mutation, the Pax2 1Neu+/- mouse, has a 30% incidence of VUR...
  3. Torres M, Gómez Pardo E, Dressler G, Gruss P. Pax-2 controls multiple steps of urogenital development. Development. 1995;121:4057-65 pubmed
    ..These data show that Pax-2 is required for multiple steps during the differentiation of intermediate mesoderm. In addition, Pax-2 mouse mutants provide an animal model for human hereditary kidney diseases...
  4. Cohen T, Loutochin O, Amin M, Capolicchio J, Goodyer P, Jednak R. PAX2 is reactivated in urinary tract obstruction and partially protects collecting duct cells from programmed cell death. Am J Physiol Renal Physiol. 2007;292:F1267-73 pubmed
    ..One such molecule, PAX2, is expressed throughout the fetal collecting duct and was recently shown to suppress apoptosis during kidney ..
  5. Jiang Y, Jiang T, Ouyang J, Zhou Q, Liang Y, Cui Y, et al. Cell atavistic transition: Paired box 2 re-expression occurs in mature tubular epithelial cells during acute kidney injury and is regulated by Angiotensin II. PLoS ONE. 2014;9:e93563 pubmed publisher
    ..In this study, we observed a transient re-expression of embryonic gene Paired box 2 (Pax2) in adult rat TECs in vivo during ischemia-reperfusion induced AKI and most Pax2 positive TECs co-expressed kidney ..
  6. Sooparb S, Price S, Shaoguang J, Franch H. Suppression of chaperone-mediated autophagy in the renal cortex during acute diabetes mellitus. Kidney Int. 2004;65:2135-44 pubmed
    ..g., M2 pyruvate kinase, glyceraldehyde-3-phosphate dehydrogenase (GAPDH) and pax2] were more abundant...
  7. Abdel Hakeem A, Henry T, Magee T, Desai M, Ross M, Mansano R, et al. Mechanisms of impaired nephrogenesis with fetal growth restriction: altered renal transcription and growth factor expression. Am J Obstet Gynecol. 2008;199:252.e1-7 pubmed publisher
    ..Maternal food restriction resulted in up-regulated mRNA expression for WT1, FGF2, and BMP7, whereas Pax2, GDNF, FGF7, BMP4, WNT4, and WNT11 mRNAs were down-regulated...
  8. Zhang S, Chen Y, Tran S, Liu F, Nestoridi E, Hébert M, et al. Pax-2 and N-myc regulate epithelial cell proliferation and apoptosis in a positive autocrine feedback loop. Pediatr Nephrol. 2007;22:813-24 pubmed
    ..Our data demonstrate that Pax-2 gene overexpression prevents hyperglycemia-induced apoptosis, and N-myc appears to provide a positive autocrine feedback on Pax-2 gene expression in embryonic mesenchymal cells. ..
  9. Brophy P, Ostrom L, Lang K, Dressler G. Regulation of ureteric bud outgrowth by Pax2-dependent activation of the glial derived neurotrophic factor gene. Development. 2001;128:4747-56 pubmed
    ..We demonstrate that Pax2 expression in the metanephric mesenchyme is independent of induction by the ureteric bud...
  10. Clarke J, Patel S, Raymond R, Andrew S, Robinson B, Dressler G, et al. Regulation of c-Ret in the developing kidney is responsive to Pax2 gene dosage. Hum Mol Genet. 2006;15:3420-8 pubmed
    During kidney development, Pax2 and Pax8 are expressed very early in the mammalian nephric duct and both precede the expression of receptor tyrosine kinase, c-Ret...
  11. Li C, Guo M, Borczuk A, Powell C, Wei M, Thaker H, et al. Gene expression in Wilms' tumor mimics the earliest committed stage in the metanephric mesenchymal-epithelial transition. Am J Pathol. 2002;160:2181-90 pubmed
    ..This signature set was enriched in genes encoding transcription factors. Four of these, PAX2, EYA1, HBF2, and HOXA11, are essential for cell survival and proliferation in early metanephric development, ..
  12. McConnell M, Cunliffe H, Chua L, Ward T, Eccles M. Differential regulation of the human Wilms tumour suppressor gene (WT1) promoter by two isoforms of PAX2. Oncogene. 1997;14:2689-700 pubmed
    b>PAX2 is a member of the paired box family of genes with an important role in kidney, genital tract and eye development. Another gene essential for kidney and genital tract development is the Wilms tumour gene, WT1...
  13. Dziarmaga A, Hueber P, Iglesias D, Hache N, Jeffs A, Gendron N, et al. Neuronal apoptosis inhibitory protein is expressed in developing kidney and is regulated by PAX2. Am J Physiol Renal Physiol. 2006;291:F913-20 pubmed
    ..Little is known about how nephron number is set. However, we previously showed that the transcription factor, Pax2, suppresses apoptosis in UB cells during kidney development and optimizes branching morphogenesis...
  14. Franch H, Sooparb S, Du J, Brown N. A mechanism regulating proteolysis of specific proteins during renal tubular cell growth. J Biol Chem. 2001;276:19126-31 pubmed
    ..EGF caused a similar increase in the half-life of the KFERQ-containing paired box-related transcription factor, Pax2. The increase in half-life was accompanied by an increased accumulation of proteins with a KFERQ motif including ..
  15. Eccles M, Yun K, Reeve A, Fidler A. Comparative in situ hybridization analysis of PAX2, PAX8, and WT1 gene transcription in human fetal kidney and Wilms' tumors. Am J Pathol. 1995;146:40-5 pubmed
    ..Two members of the paired box family of genes, PAX2 and PAX8, are expressed in WT and are potentially involved in its induction...
  16. Li L, Wu Y, Yang Y. Paired box 2 induces epithelial-mesenchymal transition in normal renal tubular epithelial cells of rats. Mol Med Rep. 2013;7:1549-54 pubmed publisher
    ..Previous studies have indicated that paired box 2 (PAX2) affects renal fibrosis, likely by regulating EMT...
  17. Mui S, Kim J, Lemke G, Bertuzzi S. Vax genes ventralize the embryonic eye. Genes Dev. 2005;19:1249-59 pubmed
  18. Hao Z, Yeung J, Wolf L, Doucette R, Nazarali A. Differential expression of Hoxa-2 protein along the dorsal-ventral axis of the developing and adult mouse spinal cord. Dev Dyn. 1999;216:201-17 pubmed dyn 1999;216:201-217. ..
  19. Zhang J, Pippin J, Vaughan M, Krofft R, Taniguchi Y, Romagnani P, et al. Retinoids augment the expression of podocyte proteins by glomerular parietal epithelial cells in experimental glomerular disease. Nephron Exp Nephrol. 2012;121:e23-37 pubmed publisher
    ..Immunohistochemistry staining of PAX2 (parietal epithelial cell marker), WT-1 (podocyte cell marker), and Ki-67 (proliferation marker) were performed on ..
  20. Barua M, Stellacci E, Stella L, Weins A, Genovese G, Muto V, et al. Mutations in PAX2 associate with adult-onset FSGS. J Am Soc Nephrol. 2014;25:1942-53 pubmed publisher members of an index family with dominant FSGS revealed a nonconservative, disease-segregating variant in the PAX2 transcription factor gene...
  21. Bose S, Gibson W, Bullard R, Donald C. PAX2 oncogene negatively regulates the expression of the host defense peptide human beta defensin-1 in prostate cancer. Mol Immunol. 2009;46:1140-8 pubmed publisher
    ..However, the factors responsible for the loss of hBD1 expression are not known. PAX2, a transcriptional regulator normally expressed during early development, has been implicated as an oncogene in ..
  22. Yuan S, Yeh Y, Lee E. Pax-2 interacts with RB and reverses its repression on the promoter of Rig-1, a Robo member. Biochem Biophys Res Commun. 2002;296:1019-25 pubmed
    ..In summary, the ubiquitous tumor suppressor RB and the neuron-enriched transcription factor Pax-2 may play a role in the regulation of Rig-1 expression during embryogenesis. ..
  23. Mae S, Shirasawa S, Yoshie S, Sato F, Kanoh Y, Ichikawa H, et al. Combination of small molecules enhances differentiation of mouse embryonic stem cells into intermediate mesoderm through BMP7-positive cells. Biochem Biophys Res Commun. 2010;393:877-82 pubmed publisher
    ..Under this condition, more than one-half of the cells were positive for Osr1, and at the same time, Pax2 (another IM marker) was detected by real-time PCR...
  24. Chen Y, Tran S, Chenier I, Chan J, Ingelfinger J, Inagami T, et al. Deficiency of intrarenal angiotensin II type 2 receptor impairs paired homeo box-2 and N-myc expression during nephrogenesis. Pediatr Nephrol. 2008;23:1769-77 pubmed publisher
    ..Deficiency of AT(2)R may impair both Pax-2 and N-myc expression, eventually resulting in glomerular hyperfiltration that may, ultimately, lead to later development of hypertension. ..
  25. Tamimi Y, Ekuere U, Laughton N, Grundy P. WNT5A is regulated by PAX2 and may be involved in blastemal predominant Wilms tumorigenesis. Neoplasia. 2008;10:1470-80 pubmed
    The PAX2 gene encodes a transcription factor expressed during development...
  26. Larsson M. Pax2 is persistently expressed by GABAergic neurons throughout the adult rat dorsal horn. Neurosci Lett. 2017;638:96-101 pubmed publisher
    The transcription factor Pax2 is required for the differentiation of GABAergic neurons in the mouse dorsal horn...
  27. Plachov D, Chowdhury K, Walther C, Simon D, Guenet J, Gruss P. Pax8, a murine paired box gene expressed in the developing excretory system and thyroid gland. Development. 1990;110:643-51 pubmed
    ..The embryonic expression pattern of Pax8 is compared with that of Pax2, another recently described paired box gene expressed in the developing excretory system.
  28. Bouchard M, de Caprona D, Busslinger M, Xu P, Fritzsch B. Pax2 and Pax8 cooperate in mouse inner ear morphogenesis and innervation. BMC Dev Biol. 2010;10:89 pubmed publisher
    b>Pax2;5;8 transcription factors play diverse roles in vertebrate and invertebrate organogenesis, including the development of the inner ear...
  29. Quinlan J, Lemire M, Hudson T, Qu H, Benjamin A, Roy A, et al. A common variant of the PAX2 gene is associated with reduced newborn kidney size. J Am Soc Nephrol. 2007;18:1915-21 pubmed
    ..In renal-coloboma syndrome, renal hypoplasia and reduced nephron number are due to heterozygous mutations of the PAX2 gene...
  30. Li L, Wu Y, Zhang W. PAX2 re-expression in renal tubular epithelial cells and correlation with renal interstitial fibrosis of rats with obstructive nephropathy. Ren Fail. 2010;32:603-11 pubmed publisher
    To investigate the effect of paired box 2 (PAX2) gene re-expression in renal tubular epithelial cells on renal interstitial fibrosis in rats with obstructive nephropathy...
  31. Torban E, Eccles M, Favor J, Goodyer P. PAX2 suppresses apoptosis in renal collecting duct cells. Am J Pathol. 2000;157:833-42 pubmed
    b>PAX2 is a transcription factor belonging to the evolutionarily conserved paired box family and is required during development of the central nervous system and genitourinary axis...
  32. Sharma P, Bowman M, Yu B, Sukumar S. A rodent model for Wilms tumors: embryonal kidney neoplasms induced by N-nitroso-N'-methylurea. Proc Natl Acad Sci U S A. 1994;91:9931-5 pubmed
    ..The histopathology of the rat kidney tumors and the genetic alterations are reminiscent of those observed in Wilms tumors, establishing this as a relevant model system for the human disease. ..
  33. Zhang S, Chen Y, Tran S, Chenier I, Hébert M, Ingelfinger J. Reactive oxygen species in the presence of high glucose alter ureteric bud morphogenesis. J Am Soc Nephrol. 2007;18:2105-15 pubmed
    ..Taken together, these data demonstrate that high glucose alters UB branching morphogenesis. This occurs, at least in part, via reactive oxygen species generation, activation of Akt signaling, and upregulation of Pax-2 gene expression. ..
  34. Sajithlal G, Zou D, Silvius D, Xu P. Eya 1 acts as a critical regulator for specifying the metanephric mesenchyme. Dev Biol. 2005;284:323-36 pubmed
    ..Using Eya 1, Gdnf, Six 1 and Pax 2 mutant mice, we show that Eya 1 probably functions at the top of the genetic hierarchy controlling kidney ..
  35. Nakane A, Kojima Y, Hayashi Y, Kohri K, Masui S, Nishinakamura R. Pax2 overexpression in embryoid bodies induces upregulation of integrin alpha8 and aquaporin-1. In Vitro Cell Dev Biol Anim. 2009;45:62-8 pubmed publisher
    The transcription factor Pax2 is essential for kidney development in mice, and overexpression of Pax2 in chick embryos leads to ectopic formation of nephric structures...
  36. Boualia S, Gaitan Y, Murawski I, Nadon R, Gupta I, Bouchard M. Vesicoureteral reflux and other urinary tract malformations in mice compound heterozygous for Pax2 and Emx2. PLoS ONE. 2011;6:e21529 pubmed publisher
    ..We found a high incidence of urinary tract anomalies in Pax2;Emx2 compound heterozygous mice that are not found in single heterozygous mice...
  37. Kobayashi H, Kawakami K, Asashima M, Nishinakamura R. Six1 and Six4 are essential for Gdnf expression in the metanephric mesenchyme and ureteric bud formation, while Six1 deficiency alone causes mesonephric-tubule defects. Mech Dev. 2007;124:290-303 pubmed
    ..The Six1/Six4-deficient metanephric mesenchyme cells were directed toward kidney lineage but failed to express Pax2, Pax8, or Gdnf, whereas the expression of these genes was partially reduced or unchanged in the case of Six1 ..
  38. Kozmik Z, Kurzbauer R, Dörfler P, Busslinger M. Alternative splicing of Pax-8 gene transcripts is developmentally regulated and generates isoforms with different transactivation properties. Mol Cell Biol. 1993;13:6024-35 pubmed
    ..Hence, alternative splicing of Pax-8 gene transcripts not only generates six different Pax-8 variants but is also temporally and spatially regulated during early mouse development. ..
  39. Donovan M, Natoli T, Sainio K, Amstutz A, Jaenisch R, Sariola H, et al. Initial differentiation of the metanephric mesenchyme is independent of WT1 and the ureteric bud. Dev Genet. 1999;24:252-62 pubmed
    ..However, the Wolffian duct from Wt1 -/- embryos was a competent inducer of wild-type metanephric mesenchyme. ..
  40. Xu H, Ou C, Rong L, Zhou T. The potential signal pathway between PAX2 and CD2AP in the renal interstitial fibrosis disease. J Recept Signal Transduct Res. 2014;34:290-8 pubmed publisher
    b>Paired box gene 2 (PAX2) can regulate tissue development and cellular differentiation, and it is associated with renal diseases...
  41. Ryan G, Steele Perkins V, Morris J, Rauscher F, Dressler G. Repression of Pax-2 by WT1 during normal kidney development. Development. 1995;121:867-75 pubmed
  42. Poleev A, Fickenscher H, Mundlos S, Winterpacht A, Zabel B, Fidler A, et al. PAX8, a human paired box gene: isolation and expression in developing thyroid, kidney and Wilms' tumors. Development. 1992;116:611-23 pubmed
    ..The truncated Pax8 protein becomes more similar to the predicted murine Pax2 protein, that is also expressed during kidney development and lacks the serine rich region...
  43. Baumer N, Marquardt T, Stoykova A, Spieler D, Treichel D, Ashery Padan R, et al. Retinal pigmented epithelium determination requires the redundant activities of Pax2 and Pax6. Development. 2003;130:2903-15 pubmed
    The transcription factors Pax2 and Pax6 are co-expressed in the entire optic vesicle (OV) prior and concomitant with the establishment of distinct neuroretinal, retinal, pigmented-epithelial and optic-stalk progenitor domains, suggesting ..
  44. Zhang S, Guo J, Moini B, Ingelfinger J. Angiotensin II stimulates Pax-2 in rat kidney proximal tubular cells: impact on proliferation and apoptosis. Kidney Int. 2004;66:2181-92 pubmed
    ..Cells lacking Pax-2 gene expression appear to be prone toward apoptosis rather than proliferation. ..
  45. Torban E, Dziarmaga A, Iglesias D, Chu L, Vassilieva T, Little M, et al. PAX2 activates WNT4 expression during mammalian kidney development. J Biol Chem. 2006;281:12705-12 pubmed
    The transcription factor PAX2 is expressed during normal kidney development and is thought to influence outgrowth and branching of the ureteric bud...
  46. Pillai A, Mansouri A, Behringer R, Westphal H, Goulding M. Lhx1 and Lhx5 maintain the inhibitory-neurotransmitter status of interneurons in the dorsal spinal cord. Development. 2007;134:357-66 pubmed well as late-born inhibitory dILA neurons (dILA), all of which express the paired-domain transcription factor Pax2. Although it appears that Lhx1 and Lhx5 do not control the initial specification of the neuronal cell types in ..
  47. Kobayashi A, Shawlot W, Kania A, Behringer R. Requirement of Lim1 for female reproductive tract development. Development. 2004;131:539-49 pubmed
    ..These studies demonstrate an essential role for Lim1 in female reproductive tract development...
  48. Dziarmaga A, Eccles M, Goodyer P. Suppression of ureteric bud apoptosis rescues nephron endowment and adult renal function in Pax2 mutant mice. J Am Soc Nephrol. 2006;17:1568-75 pubmed
    ..This study shows that the increased apoptosis and reduced ureteric bud branching of heterozygous Pax2 mutant mice is associated with 40% decrease in nephron number at birth...
  49. Mansour H, Chamberlain C, Weible M, Hughes S, Chu Y, Chan Ling T. Aging-related changes in astrocytes in the rat retina: imbalance between cell proliferation and cell death reduces astrocyte availability. Aging Cell. 2008;7:526-40 pubmed publisher
    ..Glial fibrillary acidic protein (GFAP), S100 and Pax2 were used to identify astrocytes, and blood vessels were localized using Griffonia simplicifolia isolectin B4...
  50. Alur R, Cox T, Crawford M, Gong X, Brooks B. Optic nerve axon number in mouse is regulated by PAX2. J AAPOS. 2008;12:117-21 pubmed
    Papillorenal syndrome is an autosomal-dominant disease caused by mutations in the PAX2 transcription factor gene. Patients often exhibit congenital excavation of the optic nerve and a spectrum of congenital kidney abnormalities...