Gene Symbol: Pax8
Description: paired box 8
Alias: Pax-8, paired box protein Pax-8, paired box gene 8
Species: mouse
Products:     Pax8

Top Publications

  1. Christ S, Biebel U, Hoidis S, Friedrichsen S, Bauer K, Smolders J. Hearing loss in athyroid pax8 knockout mice and effects of thyroxine substitution. Audiol Neurootol. 2004;9:88-106 pubmed
    b>Pax8-/- mice do not develop thyroid follicular structures and thus provide an ideal animal model to study the consequences of congenital hypothyroidism. Despite their athyroidism, Pax8-/- mice survive up to postnatal day 21 (P21)...
  2. Schmidt Ott K, Masckauchan T, Chen X, Hirsh B, Sarkar A, Yang J, et al. beta-catenin/TCF/Lef controls a differentiation-associated transcriptional program in renal epithelial progenitors. Development. 2007;134:3177-90 pubmed
    ..induces, in a TCF/Lef-dependent manner, a subset of the transcripts associated with epithelialization, including Pax8, cyclin D1 (Ccnd1) and Emx2...
  3. Lania G, Zhang Z, Huynh T, Caprio C, Moon A, Vitelli F, et al. Early thyroid development requires a Tbx1-Fgf8 pathway. Dev Biol. 2009;328:109-17 pubmed publisher
    ..Thus, we have established that a Tbx1->Fgf8 pathway in the pharyngeal mesoderm is a key size regulator of mammalian thyroid. ..
  4. Pfeffer P, Gerster T, Lun K, Brand M, Busslinger M. Characterization of three novel members of the zebrafish Pax2/5/8 family: dependency of Pax5 and Pax8 expression on the Pax2.1 (noi) function. Development. 1998;125:3063-74 pubmed
    The mammalian Pax2, Pax5 and Pax8 genes code for highly related transcription factors, which play important roles in embryonic development and organogenesis...
  5. 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...
  6. 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
    ..this later function, kidney development was analyzed in embryos that were compound heterozygous for Pax2 and for Pax8, a closely related member of the Pax gene family...
  7. Brandt N, Kuhn S, Münkner S, Braig C, Winter H, Blin N, et al. Thyroid hormone deficiency affects postnatal spiking activity and expression of Ca2+ and K+ channels in rodent inner hair cells. J Neurosci. 2007;27:3174-86 pubmed
    ..using patch-clamp recordings, capacitance measurements, and immunocytochemistry in hypothyroid rats and athyroid Pax8-/- mice...
  8. 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
    ..Here we describe the isolation of Pax8, a novel paired box containing clone from an 8.5 day p.c. mouse embryo cDNA library...
  9. Sendin G, Bulankina A, Riedel D, Moser T. Maturation of ribbon synapses in hair cells is driven by thyroid hormone. J Neurosci. 2007;27:3163-73 pubmed
    ..recordings of Ca2+ currents and exocytic membrane capacitance changes in IHCs of athyroid and TH-substituted Pax8-/- mice during postnatal development...

More Information


  1. Rodriguez W, Jin L, Janssens V, Pierreux C, Hick A, Urizar E, et al. Deletion of the RNaseIII enzyme dicer in thyroid follicular cells causes hypothyroidism with signs of neoplastic alterations. PLoS ONE. 2012;7:e29929 pubmed publisher
    ..of follicular cells and an ongoing de-differentiation in the center of the thyroid gland, with a loss of Pax8, FoxE1, Nis and Tpo expression...
  2. Goode D, Elgar G. The PAX258 gene subfamily: a comparative perspective. Dev Dyn. 2009;238:2951-74 pubmed publisher
    ..Members of the vertebrate PAX2, PAX5 and PAX8 gene subfamily derived from an ancient class of paired box genes and arose from such whole genome duplication ..
  3. 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
    ..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...
  4. Postiglione M, Parlato R, Rodriguez Mallon A, Rosica A, Mithbaokar P, Maresca M, et al. Role of the thyroid-stimulating hormone receptor signaling in development and differentiation of the thyroid gland. Proc Natl Acad Sci U S A. 2002;99:15462-7 pubmed
    ..are detected in the amounts of thyroglobulin and the thyroid-enriched transcription factors TTF-1, TTF-2, and Pax8. These data suggest that the major role of the TSH/TSHR pathway is in controlling genes involved in iodide ..
  5. Amendola E, De Luca P, Macchia P, Terracciano D, Rosica A, Chiappetta G, et al. A mouse model demonstrates a multigenic origin of congenital hypothyroidism. Endocrinology. 2005;146:5038-47 pubmed
    ..can be a consequence of mutations in genes encoding the TSH receptor or the transcription factors TITF1, FOXE1, or PAX8. We report here that in mouse models, the combination of partial deficiencies in the Titf1 and Pax8 genes results ..
  6. Carroll T, Park J, Hayashi S, Majumdar A, McMahon A. Wnt9b plays a central role in the regulation of mesenchymal to epithelial transitions underlying organogenesis of the mammalian urogenital system. Dev Cell. 2005;9:283-92 pubmed
    ..Together these findings suggest that Wnt9b is a common organizing signal regulating diverse components of the mammalian urogenital system...
  7. Xu P, Zheng W, Laclef C, Maire P, Maas R, Peters H, et al. Eya1 is required for the morphogenesis of mammalian thymus, parathyroid and thyroid. Development. 2002;129:3033-44 pubmed
    ..5 in Eya1(-/-) embryos. Our results indicate that Eya1 controls critical early inductive events involved in the morphogenesis of thymus, parathyroid and thyroid. ..
  8. Wright T, Mansour S. Fgf3 and Fgf10 are required for mouse otic placode induction. Development. 2003;130:3379-90 pubmed
    ..Finally, examination of embryos carrying three out of the four mutant Fgf alleles revealed intermediate phenotypes, suggesting a quantitative requirement for FGF signalling in otic vesicle formation. ..
  9. Stettner P, Bourgeois S, Marsching C, Traykova Brauch M, Porubsky S, Nordström V, et al. Sulfatides are required for renal adaptation to chronic metabolic acidosis. Proc Natl Acad Sci U S A. 2013;110:9998-10003 pubmed publisher
    ..This study points to a seminal role of sulfatides in renal ammonium handling, urinary acidification, and acid-base homeostasis. ..
  10. Riccomagno M, Martinu L, Mulheisen M, Wu D, Epstein D. Specification of the mammalian cochlea is dependent on Sonic hedgehog. Genes Dev. 2002;16:2365-78 pubmed
    ..Taken together, our data support a model whereby auditory cell fates in the otic vesicle are established by the direct action of Shh. ..
  11. Bouchard M, Souabni A, Busslinger M. Tissue-specific expression of cre recombinase from the Pax8 locus. Genesis. 2004;38:105-9 pubmed
    The transcription factor Pax8 is expressed in the developing thyroid gland, inner ear, kidney, and mid-hindbrain region. Pax8 mutant mice die only postnatally due to a thyroid gland defect...
  12. Parlato R, Rosica A, Rodriguez Mallon A, Affuso A, Postiglione M, Arra C, et al. An integrated regulatory network controlling survival and migration in thyroid organogenesis. Dev Biol. 2004;276:464-75 pubmed
    ..The experiments reported here analyze the expression of the transcription factors Titf1, Hhex, Pax8, and Foxe1 in the thyroid primordium of null mutants of each of them...
  13. Bouchard M, Pfeffer P, Busslinger M. Functional equivalence of the transcription factors Pax2 and Pax5 in mouse development. Development. 2000;127:3703-13 pubmed
    ..Together these data demonstrate that the transcription factors Pax2 and Pax5 have maintained equivalent biochemical functions since their divergence early in vertebrate evolution. ..
  14. Xu P, Adams J, Peters H, Brown M, Heaney S, Maas R. Eya1-deficient mice lack ears and kidneys and show abnormal apoptosis of organ primordia. Nat Genet. 1999;23:113-7 pubmed
    ..In addition, our results suggest that an evolutionarily conserved Pax-Eya-Six regulatory hierarchy is used in mammalian ear and kidney development. ..
  15. Zannini M, Francis Lang H, Plachov D, Di Lauro R. Pax-8, a paired domain-containing protein, binds to a sequence overlapping the recognition site of a homeodomain and activates transcription from two thyroid-specific promoters. Mol Cell Biol. 1992;12:4230-41 pubmed
    ..Thus, the promoters of thyroglobulin and thyroperoxidase represent the first identified natural targets for transcriptional activation by a paired domain-containing protein. ..
  16. Mansouri A, Chowdhury K, Gruss P. Follicular cells of the thyroid gland require Pax8 gene function. Nat Genet. 1998;19:87-90 pubmed
    ..By analysis of Pax8-/- mice, we demonstrate that Pax8 is required for the formation of the follicular cells in the thyroid...
  17. Self M, Lagutin O, Bowling B, Hendrix J, Cai Y, Dressler G, et al. Six2 is required for suppression of nephrogenesis and progenitor renewal in the developing kidney. EMBO J. 2006;25:5214-28 pubmed
    ..We propose that in the developing kidney, Six2 activity is required for maintaining the mesenchymal progenitor population in an undifferentiated state by opposing the inductive signals emanating from the ureteric bud...
  18. Fagman H, Andersson L, Nilsson M. The developing mouse thyroid: embryonic vessel contacts and parenchymal growth pattern during specification, budding, migration, and lobulation. Dev Dyn. 2006;235:444-55 pubmed
    ..It is suggested that thyroid inductive signals arise from embryonic vessels, and that this provides ideas to conceptually new pathogenetic mechanisms of thyroid dysgenesis. ..
  19. Di Palma T, Nitsch R, Mascia A, Nitsch L, Di Lauro R, Zannini M. The paired domain-containing factor Pax8 and the homeodomain-containing factor TTF-1 directly interact and synergistically activate transcription. J Biol Chem. 2003;278:3395-402 pubmed
    Pax genes encode for transcription factors essential for tissue development in many species. Pax8, the only member of the family expressed in the thyroid tissue, is involved in the morphogenesis of the gland and in the transcriptional ..
  20. Frezzetti D, Reale C, Cali G, Nitsch L, Fagman H, Nilsson O, et al. The microRNA-processing enzyme Dicer is essential for thyroid function. PLoS ONE. 2011;6:e27648 pubmed publisher
  21. Bouchard M, Souabni A, Mandler M, Neubüser A, Busslinger M. Nephric lineage specification by Pax2 and Pax8. Genes Dev. 2002;16:2958-70 pubmed
    ..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...
  22. Yang D, Lai D, Huang X, Shi X, Gao Z, Huang F, et al. The defects in development and apoptosis of cardiomyocytes in mice lacking the transcriptional factor Pax-8. Int J Cardiol. 2012;154:43-51 pubmed publisher
    ..Knockout of Pax-8 may exert a similar effect on myocardial morphology and apoptosis as those seen in ALK3 knockouts. Furthermore, the ventricular septum malformations could be partially attributed to accelerated cardiomyocyte apoptosis. ..
  23. Ranghini E, Dressler G. Evidence for intermediate mesoderm and kidney progenitor cell specification by Pax2 and PTIP dependent mechanisms. Dev Biol. 2015;399:296-305 pubmed publisher
    ..These data provide evidence that Pax2 specifies the intermediate mesoderm and renal epithelial cells through epigenetic mechanisms and in part by repressing paraxial mesodermal fate. ..
  24. Mukhopadhyay D, Plateroti M, Anant S, Nassir F, Samarut J, Davidson N. Thyroid hormone regulates hepatic triglyceride mobilization and apolipoprotein B messenger ribonucleic Acid editing in a murine model of congenital hypothyroidism. Endocrinology. 2003;144:711-9 pubmed
    ..hormone-dependent regulation of apolipoprotein B (apoB) RNA editing in a strain of congenitally hypothyroid mice (Pax8(-/-)) that lacks thyroid follicular cells...
  25. Nitsch R, Di Dato V, di Gennaro A, De Cristofaro T, Abbondante S, De Felice M, et al. Comparative genomics reveals a functional thyroid-specific element in the far upstream region of the PAX8 gene. BMC Genomics. 2010;11:306 pubmed publisher
    ..It is also well known that Pax8, TTF-1, Foxe1 and Hhex are the thyroid-enriched transcription factors responsible for the expression of the above ..
  26. Zannini M, Avantaggiato V, Biffali E, Arnone M, Sato K, Pischetola M, et al. TTF-2, a new forkhead protein, shows a temporal expression in the developing thyroid which is consistent with a role in controlling the onset of differentiation. EMBO J. 1997;16:3185-97 pubmed
  27. Mao Y, Mulvaney J, Zakaria S, Yu T, Morgan K, Allen S, et al. Characterization of a Dchs1 mutant mouse reveals requirements for Dchs1-Fat4 signaling during mammalian development. Development. 2011;138:947-57 pubmed publisher
    ..Our analysis implies that Dchs1 and Fat4 function as a ligand-receptor pair during murine development, and identifies novel requirements for Dchs1-Fat4 signaling in multiple organs. ..
  28. Poleev A, Wendler F, Fickenscher H, Zannini M, Yaginuma K, Abbott C, et al. Distinct functional properties of three human paired-box-protein, PAX8, isoforms generated by alternative splicing in thyroid, kidney and Wilms' tumors. Eur J Biochem. 1995;228:899-911 pubmed
    ..The murine and human Pax8 genes are expressed in developing and adult thyroid as well as in the developing secretory system and at the lower ..
  29. Berry R, Ozdemir D, Aronow B, Lindström N, Dudnakova T, Thornburn A, et al. Deducing the stage of origin of Wilms' tumours from a developmental series of Wt1-mutant mice. Dis Model Mech. 2015;8:903-17 pubmed publisher
  30. Spina A, Rea S, De Pasquale V, Mastellone V, Avallone L, Pavone L. Fate map of serotonin transporter-expressing cells in developing mouse thyroid. Anat Rec (Hoboken). 2011;294:384-90 pubmed publisher
    ..The co-localization of lacZ staining in the same cells that express the transcription factors Nkx2.1 and Pax8 at the E12.5 stage confirms their identity as thyroid cell precursors...
  31. Loikkanen I, Lin Y, Railo A, Pajunen A, Vainio S. Polyamines are involved in murine kidney development controlling expression of c-ret, E-cadherin, and Pax2/8 genes. Differentiation. 2005;73:303-12 pubmed
    ..We propose that ODC and polyamines have an important role in kidney organogenesis, being involved in the regulation of the expression of genes implicated in epithelial-mesenchymal tissue interactions. ..
  32. Kiefer S, Robbins L, Rauchman M. Conditional expression of Wnt9b in Six2-positive cells disrupts stomach and kidney function. PLoS ONE. 2012;7:e43098 pubmed publisher
    ..These results demonstrate that expression of Wnt9b in Six2-positive cells disrupts cell fate decisions in the kidney and the gastrointestinal tract...
  33. Kariyawasam D, Rachdi L, Carré A, Martin M, Houlier M, Janel N, et al. DYRK1A BAC transgenic mouse: a new model of thyroid dysgenesis in Down syndrome. Endocrinology. 2015;156:1171-80 pubmed publisher
    ..The expression of transcription factors Nkx2-1, Pax8, and Foxe1 involved in thyroidogenesis were studied by quantitative RT-PCR at the same embryonic stages...
  34. Kersseboom S, Horn S, Visser W, Chen J, Friesema E, Vaurs Barrière C, et al. In vitro and mouse studies supporting therapeutic utility of triiodothyroacetic acid in MCT8 deficiency. Mol Endocrinol. 2014;28:1961-70 pubmed publisher
    ..In transfected cells, TA3 did not show significant transport by MCT8. Most importantly, treatment of athyroid Pax8-knockout mice and Mct8/Oatp1c1-double knockout mice between postnatal days 1 and 12 with TA3 restored T3-dependent ..
  35. Moser M, Pscherer A, Roth C, Becker J, Mücher G, Zerres K, et al. Enhanced apoptotic cell death of renal epithelial cells in mice lacking transcription factor AP-2beta. Genes Dev. 1997;11:1938-48 pubmed
    ..Sequence analyses of ARPKD patients and linkage analyses using intragenic polymorphic markers indicate that the AP-2beta gene is located in close proximity to but distinct from the ARPKD gene. ..
  36. Alfred J, Rance K, Taylor B, Phillips S, Abbott C, Jackson I. Mapping in the region of Danforth's short tail and the localization of tail length modifiers. Genome Res. 1997;7:108-17 pubmed
    ..This has allowed us to identify a major QTL on chromosome 10 in the region of nodal and three other putative tail length QTL on chromosomes 1, 9, and 18. ..
  37. 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
    ..As both genes are located on human chromosome 10q, which is lost in a proportion of VUR patients, these findings may help understand VUR and CAKUT in humans. ..
  38. Fagman H, Amendola E, Parrillo L, Zoppoli P, Marotta P, Scarfò M, et al. Gene expression profiling at early organogenesis reveals both common and diverse mechanisms in foregut patterning. Dev Biol. 2011;359:163-75 pubmed publisher
    ..As an initial step in this direction we describe a regulatory pathway involving the anti-apoptotic gene Bcl2 that controls cell survival in early thyroid development. ..
  39. Amendola E, Sanges R, Galvan A, Dathan N, Manenti G, Ferrandino G, et al. A locus on mouse chromosome 2 is involved in susceptibility to congenital hypothyroidism and contains an essential gene expressed in thyroid. Endocrinology. 2010;151:1948-58 pubmed publisher
    ..development of congenital hypothyroidism in mice heterozygous for null mutations in genes encoding Nkx2-1/Titf1 and Pax8. The two strains showing a differential predisposition to congenital hypothyroidism contain several single-..
  40. Vázquez Echeverría C, Dominguez Frutos E, Charnay P, Schimmang T, Pujades C. Analysis of mouse kreisler mutants reveals new roles of hindbrain-derived signals in the establishment of the otic neurogenic domain. Dev Biol. 2008;322:167-78 pubmed publisher
    ..These results highlight the importance of hindbrain-derived signals in the regulation of otic neurogenesis. ..
  41. Carre A, Rachdi L, Tron E, Richard B, Castanet M, Schlumberger M, et al. Hes1 is required for appropriate morphogenesis and differentiation during mouse thyroid gland development. PLoS ONE. 2011;6:e16752 pubmed publisher
  42. Veikkolainen V, Naillat F, Railo A, Chi L, Manninen A, Hohenstein P, et al. ErbB4 modulates tubular cell polarity and lumen diameter during kidney development. J Am Soc Nephrol. 2012;23:112-22 pubmed publisher
    ..We examined ErbB4 function in developing kidney tubules in vivo with Pax8-Cre-mediated conditional overexpression of Rosa26 locus-targeted ERBB4 and with conditional Erbb4 knock-out mice...
  43. Nitsch R, Di Palma T, Mascia A, Zannini M. WBP-2, a WW domain binding protein, interacts with the thyroid-specific transcription factor Pax8. Biochem J. 2004;377:553-60 pubmed means of immunological screening of the WW domain binding protein WBP-2 as a biochemical interactor of Pax8 (a WW domain is a protein-interaction domain containing two conserved tryptophan residues)...
  44. Friedrichsen S, Christ S, Heuer H, Schafer M, Mansouri A, Bauer K, et al. Regulation of iodothyronine deiodinases in the Pax8-/- mouse model of congenital hypothyroidism. Endocrinology. 2003;144:777-84 pubmed
    ..As an animal model for CHT, we studied Pax8(-/-) mice, which are born without a thyroid gland...
  45. Adams B, Dörfler P, Aguzzi A, Kozmik Z, Urbanek P, Maurer Fogy I, et al. Pax-5 encodes the transcription factor BSAP and is expressed in B lymphocytes, the developing CNS, and adult testis. Genes Dev. 1992;6:1589-607 pubmed
    ..All of this evidence indicates that the transcription factor BSAP may not only play an important role in B-cell differentiation but also in neural development and spermatogenesis. ..
  46. Barrionuevo F, Naumann A, Bagheri Fam S, Speth V, Taketo M, Scherer G, et al. Sox9 is required for invagination of the otic placode in mice. Dev Biol. 2008;317:213-24 pubmed publisher
  47. Westerlund J, Andersson L, Carlsson T, Zoppoli P, Fagman H, Nilsson M. Expression of Islet1 in thyroid development related to budding, migration, and fusion of primordia. Dev Dyn. 2008;237:3820-9 pubmed publisher
    ..5. In Isl1 null mutants thyroid progenitors expressing Nkx2.1 and Pax8 were readily specified in the anterior endoderm but the size of the thyroid rudiment was reduced...
  48. Hirata H, Tomita K, Bessho Y, Kageyama R. Hes1 and Hes3 regulate maintenance of the isthmic organizer and development of the mid/hindbrain. EMBO J. 2001;20:4454-66 pubmed
    ..These results indicate that Hes1 and Hes3 prevent premature differentiation and maintain the organizer activity of the isthmic cells, thereby regulating the development of the midbrain and anterior hindbrain. ..
  49. Chiang P, Wang S, Smithivas P, Song W, Crombez E, Akhtar A, et al. Isolation and characterization of the human and mouse homologues (SUPT4H and Supt4h) of the yeast SPT4 gene. Genomics. 1996;34:368-75 pubmed
    ..g., such as most histones), in all fetal and adult tissues that we examined. ..
  50. 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
    ..There is no detectable morphological defect in Pax8 null mice suggesting that Pax2 expression can compensate for Pax8...
  51. Dudley A, Lyons K, Robertson E. A requirement for bone morphogenetic protein-7 during development of the mammalian kidney and eye. Genes Dev. 1995;9:2795-807 pubmed
    ..Consequently, homozygous mutant animals exhibit renal dysplasia and anophthalmia at birth. Overall, these findings identify BMP-7 as an essential signaling molecule during mammalian kidney and eye development. ..
  52. De Cristofaro T, Di Palma T, Fichera I, Lucci V, Parrillo L, De Felice M, et al. An essential role for Pax8 in the transcriptional regulation of cadherin-16 in thyroid cells. Mol Endocrinol. 2012;26:67-78 pubmed publisher
    ..finding prompted us to note that the expression profile of Cadherin-16 resembles that of the transcription factor Pax8, a member of the Pax (paired-box) gene family, predominantly expressed in the developing and adult kidney and ..
  53. Xu J, Liu H, Park J, Lan Y, Jiang R. Osr1 acts downstream of and interacts synergistically with Six2 to maintain nephron progenitor cells during kidney organogenesis. Development. 2014;141:1442-52 pubmed publisher
  54. Reale C, Iervolino A, Scudiero I, Ferravante A, D Andrea L, Mazzone P, et al. NF-κB Essential Modulator (NEMO) Is Critical for Thyroid Function. J Biol Chem. 2016;291:5765-73 pubmed publisher
    ..Thus, NEMO-dependent signaling is essential for normal thyroid physiology. ..
  55. Rodgers L, O Hainmhire E, Young A, Burdette J. Loss of PAX8 in high-grade serous ovarian cancer reduces cell survival despite unique modes of action in the fallopian tube and ovarian surface epithelium. Oncotarget. 2016;7:32785-95 pubmed publisher
    High-grade serous carcinoma (HGSC) is the most common and lethal form of ovarian cancer. PAX8 is a transcription factor expressed in fallopian tube epithelial cells and in 80-96% of HGSC tumors...
  56. Nishiguchi Y, Ohmoto M, Koki J, Enomoto T, Kominami R, Matsumoto I, et al. Bcl11b/Ctip2 is required for development of lingual papillae in mice. Dev Biol. 2016;416:98-110 pubmed publisher
    ..These results indicate that Bcl11b regulates the differentiation of keratinocytes in the tongue and identify Bcl11b as an essential factor for the lingual papilla morphogenesis. ..
  57. Mittag J, Friedrichsen S, Strube A, Heuer H, Bauer K. Analysis of hypertrophic thyrotrophs in pituitaries of athyroid Pax8-/- mice. Endocrinology. 2009;150:4443-9 pubmed publisher
    Thyroid hormone is important for pituitary development and maintenance. We previously reported that in the Pax8(-/-) mouse model of congenital hypothyroidism, lactotrophs are almost undetectable, whereas the thyrotrophs exhibit ..
  58. Sellitti D, Puggina E, Lagranha C, Doi S, Pithon Curi T, Kohn L, et al. TGF-beta-like transcriptional effects of thyroglobulin (Tg) in mouse mesangial cells. Endocr J. 2007;54:449-58 pubmed
    ..These results suggest that Tg and other proteins could initiate glomerular injury by reproducing the actions of TGF-beta1 in the mesangial cell. ..
  59. Stapleton P, Weith A, Urbanek P, Kozmik Z, Busslinger M. Chromosomal localization of seven PAX genes and cloning of a novel family member, PAX-9. Nat Genet. 1993;3:292-8 pubmed
    ..PAX-1 and PAX-7 map to chromosomal regions containing previously assigned disease loci. ..
  60. 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
    ..we have found a cell-autonomous requirement for either Lhx1 or Lhx5 to maintain the expression of Pax2, Pax5 and Pax8 in dorsal inhibitory neurons at later developmental stages...
  61. Friedrichsen S, Christ S, Heuer H, Schafer M, Parlow A, Visser T, et al. Expression of pituitary hormones in the Pax8-/- mouse model of congenital hypothyroidism. Endocrinology. 2004;145:1276-83 pubmed
    ..b>Pax8-/- mice, which are born without a thyroid gland, were used to study the influence of thyroid hormones on the ..
  62. Boualia S, Gaitan Y, Tremblay M, Sharma R, Cardin J, Kania A, et al. A core transcriptional network composed of Pax2/8, Gata3 and Lim1 regulates key players of pro/mesonephros morphogenesis. Dev Biol. 2013;382:555-66 pubmed publisher
  63. Mansouri A, Goudreau G, Gruss P. Pax genes and their role in organogenesis. Cancer Res. 1999;59:1707s-1709s; discussion 1709s-1710s pubmed
    ..In particular, they are involved in the regulation of early steps in organ development. They act to define the regional specification of distinct germ layers. ..
  64. Flamant F, Poguet A, Plateroti M, Chassande O, Gauthier K, Streichenberger N, et al. Congenital hypothyroid Pax8(-/-) mutant mice can be rescued by inactivating the TRalpha gene. Mol Endocrinol. 2002;16:24-32 pubmed
    Mice devoid of all TRs are viable, whereas Pax8(-/-) mice, which lack the follicular cells producing T4 and T3 in the thyroid gland, die during the first weeks of postnatal life...
  65. Wendl T, Lun K, Mione M, Favor J, Brand M, Wilson S, et al. Pax2.1 is required for the development of thyroid follicles in zebrafish. Development. 2002;129:3751-60 pubmed
    ..We show that pax2.1 and pax8, members of the zebrafish pax2/5/8 paralogue group, are expressed in the thyroid primordium...
  66. Marotta P, Amendola E, Scarfò M, De Luca P, Zoppoli P, Amoresano A, et al. The paired box transcription factor Pax8 is essential for function and survival of adult thyroid cells. Mol Cell Endocrinol. 2014;396:26-36 pubmed publisher
    The transcription factor Pax8 is already known to be essential at very early stages of mouse thyroid gland development, before the onset of thyroid hormone production...
  67. Poguet A, Legrand C, Feng X, Yen P, Meltzer P, Samarut J, et al. Microarray analysis of knockout mice identifies cyclin D2 as a possible mediator for the action of thyroid hormone during the postnatal development of the cerebellum. Dev Biol. 2003;254:188-99 pubmed
    ..We used microarray analysis to identify new target genes in brain. Thyroid hormone treatment of hypothyroid Pax8(-/-) knockout mice, which lack thyroid follicular cells, had a very limited global effect on brain transcripts...
  68. Antonica F, Kasprzyk D, Opitz R, Iacovino M, Liao X, Dumitrescu A, et al. Generation of functional thyroid from embryonic stem cells. Nature. 2012;491:66-71 pubmed publisher
    ..Here we report that a transient overexpression of the transcription factors NKX2-1 and PAX8 is sufficient to direct mouse embryonic stem-cell differentiation into thyroid follicular cells that organize into ..
  69. Ladher R, Wright T, Moon A, Mansour S, Schoenwolf G. FGF8 initiates inner ear induction in chick and mouse. Genes Dev. 2005;19:603-13 pubmed
    ..Thus, Fgf8 plays a critical upstream role in an FGF signaling cascade required for otic induction in chick and mouse. ..
  70. Wistuba J, Mittag J, Luetjens C, Cooper T, Yeung C, Nieschlag E, et al. Male congenital hypothyroid Pax8-/- mice are infertile despite adequate treatment with thyroid hormone. J Endocrinol. 2007;192:99-109 pubmed
    ..In this study, Pax8(-/-) mice that are born without a thyroid gland were used as an animal model to study the consequences of ..
  71. 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
    ..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 deficiency...
  72. Bielesz B, Sirin Y, Si H, Niranjan T, Gruenwald A, Ahn S, et al. Epithelial Notch signaling regulates interstitial fibrosis development in the kidneys of mice and humans. J Clin Invest. 2010;120:4040-54 pubmed publisher
    ..In summary, our experiments establish that epithelial injury and Notch signaling play key roles in fibrosis development and indicate that Notch blockade may be a therapeutic strategy to reduce fibrosis and ESRD development. ..
  73. Hick A, Delmarcelle A, Bouquet M, Klotz S, Copetti T, Forez C, et al. Reciprocal epithelial:endothelial paracrine interactions during thyroid development govern follicular organization and C-cells differentiation. Dev Biol. 2013;381:227-40 pubmed publisher
    ..In turn, endothelial cells control epithelial reorganization in follicles and C-cells differentiation. ..
  74. Mansouri A, Stoykova A, Gruss P. Pax genes in development. J Cell Sci Suppl. 1994;18:35-42 pubmed
    ..To date three Pax genes have been shown to be mutated in undulated, Splotch and small eye, respectively. In man, Pax-3 is mutated in the Waardenburg syndrome, while in aniridia Pax-6 is mutated. ..
  75. 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
    Recent evidence indicates a crucial role for paired box genes in mouse and human embryogenesis. The murine Pax8 gene encodes a sequence-specific transcription factor and is expressed in the developing secretory system as well as in the ..
  76. Asano M, Gruss P. Pax-5 is expressed at the midbrain-hindbrain boundary during mouse development. Mech Dev. 1992;39:29-39 pubmed
    ..Unlike Pax-2 and Pax-8, Pax-5 was not expressed in the developing excretory system, thyroid, eye or ear. Our data suggest that Pax-5 has a role in the development of the central nervous system. ..