Gene Symbol: Prrx1
Description: paired related homeobox 1
Alias: A230024N07Rik, AA755424, AI385634, AI843499, Pmx, Pmx1, Prx1, mHox, paired mesoderm homeobox protein 1, homeobox protein K-2, homeobox protein mHox, paired-related homeobox protein 1
Species: mouse
Products:     Prrx1

Top Publications

  1. Leussink B, Brouwer A, El Khattabi M, Poelmann R, Gittenberger de Groot A, Meijlink F. Expression patterns of the paired-related homeobox genes MHox/Prx1 and S8/Prx2 suggest roles in development of the heart and the forebrain. Mech Dev. 1995;52:51-64 pubmed
    Prx1 and Prx2 (previously called MHox and S8, respectively) are the members of a small subfamily of vertebrate homeobox genes expressed during embryogenesis from gastrulation onwards...
  2. Martin J, Olson E. Identification of a prx1 limb enhancer. Genesis. 2000;26:225-9 pubmed
    Mice with a loss of function of prx1, a paired-related homeobox gene formerly called Mhox, showed craniofacial defects, limb shortening, and incompletely penetrant spina bifida...
  3. Martin J, Bradley A, Olson E. The paired-like homeo box gene MHox is required for early events of skeletogenesis in multiple lineages. Genes Dev. 1995;9:1237-49 pubmed
    ..b>MHox is a homeo box-containing gene that is expressed in the mesenchyme of facial, limb, and vertebral skeletal ..
  4. ten Berge D, Brouwer A, Korving J, Reijnen M, van Raaij E, Verbeek F, et al. Prx1 and Prx2 are upstream regulators of sonic hedgehog and control cell proliferation during mandibular arch morphogenesis. Development. 2001;128:2929-38 pubmed
    The aristaless-related homeobox genes Prx1 and Prx2 are required for correct skeletogenesis in many structures...
  5. Lu M, Cheng H, Kern M, Potter S, Tran B, Diekwisch T, et al. prx-1 functions cooperatively with another paired-related homeobox gene, prx-2, to maintain cell fates within the craniofacial mesenchyme. Development. 1999;126:495-504 pubmed
  6. Bergwerff M, Gittenberger de Groot A, Wisse L, DeRuiter M, Wessels A, Martin J, et al. Loss of function of the Prx1 and Prx2 homeobox genes alters architecture of the great elastic arteries and ductus arteriosus. Virchows Arch. 2000;436:12-9 pubmed
    Prx1 (MHox) and Prx2 (S8) are non-clustered homeobox genes that are expressed in a complex, mostly mesenchyme-specific pattern throughout embryogenesis...
  7. Chesterman E, Gainey G, Varn A, Peterson R, Kern M. Investigation of Prx1 protein expression provides evidence for conservation of cardiac-specific posttranscriptional regulation in vertebrates. Dev Dyn. 2001;222:459-70 pubmed
    Gene targeting experiments have defined that the homeobox gene Prx1 is essential for normal craniofacial, limb, and vascular development...
  8. Lu M, Pressman C, Dyer R, Johnson R, Martin J. Function of Rieger syndrome gene in left-right asymmetry and craniofacial development. Nature. 1999;401:276-8 pubmed
    ..These data reveal a critical role for pitx2 in left-right asymmetry but indicate that pitx2 may function at an intermediate step in cardiac morphogenesis and embryonic rotation. ..
  9. ten Berge D, Brouwer A, Korving J, Martin J, Meijlink F. Prx1 and Prx2 in skeletogenesis: roles in the craniofacial region, inner ear and limbs. Development. 1998;125:3831-42 pubmed
    b>Prx1 and Prx2 are closely related paired-class homeobox genes that are expressed in very similar patterns predominantly in mesenchyme. Prx1 loss-of-function mutants show skeletal defects in skull, limbs and vertebral column (Martin, J. F...

More Information


  1. Chattopadhyay A, Pinkaew D, Doan H, Jacob R, Verma S, Friedman H, et al. Fortilin potentiates the peroxidase activity of Peroxiredoxin-1 and protects against alcohol-induced liver damage in mice. Sci Rep. 2016;6:18701 pubmed publisher
    ..Here we report that fortilin physically interacts with the antioxidant enzyme peroxiredoxin-1 (PRX1), protects it from proteasome-mediated degradation, and keeps it enzymatically active by blocking its deactivating ..
  2. Morroll S, Goodchild M, Salmon N, Copeland N, Gilbert D, Jenkins N, et al. The genes encoding E-selectin (SELE) and lymphotactin (SCYC1) lie on separate chicken chromosomes although they are closely linked in human and mouse. Immunogenetics. 2001;53:477-82 pubmed
    ..In the mouse, we demonstrate that Sele is flanked by Prrx1 (paired-related homeobox gene 1) and Scyc1 and define the order of, and distances between, loci as centromere-..
  3. Shang Y, Yoshida T, Amendt B, Martin J, Owens G. Pitx2 is functionally important in the early stages of vascular smooth muscle cell differentiation. J Cell Biol. 2008;181:461-73 pubmed publisher
    ..reduces expression of SMC differentiation marker genes in the early stages of SMC differentiation in vitro, whereas Prx1, another homeodomain protein, regulates SMC differentiation marker genes in fully differentiated SMCs...
  4. Lee Y, Song D, Yoo J, Hyung K, Lee M, Moon Y, et al. Protective functions of peroxiredoxin-1 against cytokine-induced MIN6 pancreatic ?-cell line death. Can J Physiol Pharmacol. 2013;91:1037-43 pubmed publisher
    ..Overall, the results of this study suggest possible protective roles of the antioxidant enzyme PRX-1 in the insulinoma cell line MIN6 and possibly in pancreatic ?-cells under T1D conditions. ..
  5. Compagnucci C, Debiais Thibaud M, Coolen M, Fish J, Griffin J, Bertocchini F, et al. Pattern and polarity in the development and evolution of the gnathostome jaw: both conservation and heterotopy in the branchial arches of the shark, Scyliorhinus canicula. Dev Biol. 2013;377:428-48 pubmed publisher
  6. Cong Q, Jia H, Biswas S, Li P, Qiu S, Deng Q, et al. p38α MAPK Regulates Lineage Commitment and OPG Synthesis of Bone Marrow Stromal Cells to Prevent Bone Loss under Physiological and Pathological Conditions. Stem Cell Reports. 2016;6:566-578 pubmed publisher
    ..Yet how BM-MSC lineage commitment is regulated remains unclear. We show that ablation of p38α in Prx1+ BM-MSCs produced osteoporotic phenotypes, growth plate defects, and increased bone marrow fat, secondary to biased ..
  7. McGlinn E, van Bueren K, Fiorenza S, Mo R, Poh A, Forrest A, et al. Pax9 and Jagged1 act downstream of Gli3 in vertebrate limb development. Mech Dev. 2005;122:1218-33 pubmed
    ..Our data have also revealed that perturbation of early patterning events within the Gli3(Xt/Xt) limb culminates in a specific delay of anterior chondrogenesis which is subsequently realised as extra digits. ..
  8. Chesterman E, Kern M. Comparative analysis of Prx1 and Prx2 expression in mice provides evidence for incomplete compensation. Anat Rec. 2002;266:1-4 pubmed
    The family of paired-related homeobox genes to which Prx1 and Prx2 belong plays an integral role in limb and craniofacial development, as evidenced by both transgenic mice and in situ hybridization data...
  9. Beverdam A, Meijlink F. Expression patterns of group-I aristaless-related genes during craniofacial and limb development. Mech Dev. 2001;107:163-7 pubmed
  10. Vickerman L, Neufeld S, Cobb J. Shox2 function couples neural, muscular and skeletal development in the proximal forelimb. Dev Biol. 2011;350:323-36 pubmed publisher
    ..These data demonstrate that Shox2 is required for normal skeletal, neural and muscular development in the forelimb at a similar early developmental stage in each tissue. ..
  11. Brugger S, Merrill A, Torres Vazquez J, Wu N, Ting M, Cho J, et al. A phylogenetically conserved cis-regulatory module in the Msx2 promoter is sufficient for BMP-dependent transcription in murine and Drosophila embryos. Development. 2004;131:5153-65 pubmed
  12. Hudson R, Taniguchi Sidle A, Boras K, Wiggan O, Hamel P. Alx-4, a transcriptional activator whose expression is restricted to sites of epithelial-mesenchymal interactions. Dev Dyn. 1998;213:159-69 pubmed
    ..Taken together, our results demonstrate that Alx-4 is a potent transcriptional activator that is expressed at sites of epithelial-mesenchymal interactions during murine embryonic development. ..
  13. Bildsoe H, Fan X, Wilkie E, Ashoti A, Jones V, Power M, et al. Transcriptional targets of TWIST1 in the cranial mesoderm regulate cell-matrix interactions and mesenchyme maintenance. Dev Biol. 2016;418:189-203 pubmed publisher
  14. Wilk K, Yeh S, Mortensen L, Ghaffarigarakani S, Lombardo C, Bassir S, et al. Postnatal Calvarial Skeletal Stem Cells Expressing PRX1 Reside Exclusively in the Calvarial Sutures and Are Required for Bone Regeneration. Stem Cell Reports. 2017;8:933-946 pubmed publisher
    Post-natal skeletal stem cells expressing PRX1 (pnPRX1+) have been identified in the calvaria and in the axial skeleton. Here we characterize the location and functional capacity of the calvarial pnPRX1+ cells...
  15. Cohen E, Ihida Stansbury K, Lu M, Panettieri R, Jones P, Morrisey E. Wnt signaling regulates smooth muscle precursor development in the mouse lung via a tenascin C/PDGFR pathway. J Clin Invest. 2009;119:2538-49 pubmed publisher
    ..Together, these data define a Wnt/Tnc/Pdgfr signaling axis that is critical for smooth muscle development and disease progression in the lung. ..
  16. Reichert M, Takano S, von Burstin J, Kim S, Lee J, Ihida Stansbury K, et al. The Prrx1 homeodomain transcription factor plays a central role in pancreatic regeneration and carcinogenesis. Genes Dev. 2013;27:288-300 pubmed publisher
    ..ADM, and the progression of normal cells to PanIN lesions, we undertook a systematic approach to identify the Prrx1 paired homeodomain Prrx1 transcriptional factor as a highly regulated gene in these processes...
  17. Lours Calet C, Alvares L, El Hanfy A, Gandesha S, Walters E, Sobreira D, et al. Evolutionarily conserved morphogenetic movements at the vertebrate head-trunk interface coordinate the transport and assembly of hypopharyngeal structures. Dev Biol. 2014;390:231-46 pubmed publisher
    ..Thus, we have discovered evolutionarily conserved morphogenetic movements, driven by the occipital lateral mesoderm and ectoderm, that ensure cell transport and organ assembly at the head-trunk interface. ..
  18. Conway S, Henderson D, Copp A. Pax3 is required for cardiac neural crest migration in the mouse: evidence from the splotch (Sp2H) mutant. Development. 1997;124:505-14 pubmed
    ..due to down-regulation of Pax3 in neural crest cells, as use of independent neural crest markers, Hoxa-3, CrabpI, Prx1, Prx2 and c-met also revealed a deficiency of migrating cardiac neural crest cells in homozygous embryos...
  19. Depew M, Lufkin T, Rubenstein J. Specification of jaw subdivisions by Dlx genes. Science. 2002;298:381-5 pubmed
    ..We suggest that nested Dlx expression in the arches patterns their proximodistal axes. Evolutionary acquisition and subsequent refinement of jaws may have been dependent on modification of Dlx expression. ..
  20. Tavares A, Cox T, Maxson R, Ford H, Clouthier D. Negative regulation of endothelin signaling by SIX1 is required for proper maxillary development. Development. 2017;144:2021-2031 pubmed publisher
    ..Together, our results illustrate that SIX1 is the central mediator of dorsal mandibular arch identity, thus ensuring separation of bone development between the upper and lower jaws. ..
  21. Johnstone C, White S, Tebbutt N, Clay F, Ernst M, Biggs W, et al. Analysis of the regulation of the A33 antigen gene reveals intestine-specific mechanisms of gene expression. J Biol Chem. 2002;277:34531-9 pubmed
    ..Taken together, our data lead us to propose that the activity of CDX1 is pivotal in mediating the exquisite, intestine-specific expression pattern of the A33 antigen gene. ..
  22. Lu M, Cheng H, Lacy A, Kern M, Argao E, Potter S, et al. Paired-related homeobox genes cooperate in handplate and hindlimb zeugopod morphogenesis. Dev Biol. 1999;205:145-57 pubmed
    ..These data suggest that prx-1 and prx-2 cooperatively regulate handplate and hindlimb zeugopod morphogenesis through BMP-mediated signaling pathways. ..
  23. Lee K, Park J, Kim Y, Soo Lee Y, Sook Hwang T, Kim D, et al. Differential expression of Prx I and II in mouse testis and their up-regulation by radiation. Biochem Biophys Res Commun. 2002;296:337-42 pubmed
    ..Taken together, we suggest that the relative radiation-resistance of Leydig and Sertoli cells could be attributed in part to the antioxidant function of the Prx system in these cells. ..
  24. Shimozaki K, Clemenson G, Gage F. Paired related homeobox protein 1 is a regulator of stemness in adult neural stem/progenitor cells. J Neurosci. 2013;33:4066-75 pubmed publisher
    ..We show here that paired related homeobox protein Prx1 (MHox1/Prrx1) plays an important role in the maintenance of adult NSCs...
  25. Marikawa Y, Fujita T, Alarcon V. An enhancer-trap LacZ transgene reveals a distinct expression pattern of Kinesin family 26B in mouse embryos. Dev Genes Evol. 2004;214:64-71 pubmed
    ..5-kb mRNA was the major Kif26B transcript in the embryo, it was absent in many adult tissues. These results imply that KIF26B may play a role in embryogenesis, specifically in the development of limbs, face, and somites. ..
  26. Iakoubova O, Pacella L, Her H, Beier D. LTW4 protein on mouse chromosome 1 is a member of a family of antioxidant proteins. Genomics. 1997;42:474-8 pubmed
    ..The murine MER5 gene is also a member of this gene family and has recently been renamed Antioxidant protein 1 (Aop1), based on its functional characterization. We therefore propose that the gene encoding LTW4 be called Aop2. ..
  27. Tribioli C, Robledo R, Lufkin T. The murine fork head gene Foxn2 is expressed in craniofacial, limb, CNS and somitic tissues during embryogenesis. Mech Dev. 2002;118:161-3 pubmed
    ..The dynamic and specific expression of Foxn2 during embryonic development suggest multiple independent roles for Foxn2 function during gestation. ..
  28. Uwayama J, Hirayama A, Yanagawa T, Warabi E, Sugimoto R, Itoh K, et al. Tissue Prx I in the protection against Fe-NTA and the reduction of nitroxyl radicals. Biochem Biophys Res Commun. 2006;339:226-31 pubmed
    ..These results demonstrate that Prx I-/- mice have less reducing activity and are more susceptible to the damage mediated by reactive oxygen species in vivo than wild-type mice. ..
  29. Lin S, Tzeng B, Lee K, Smith R, Campbell K, Chen C. Cav3.2 T-type calcium channel is required for the NFAT-dependent Sox9 expression in tracheal cartilage. Proc Natl Acad Sci U S A. 2014;111:E1990-8 pubmed publisher
    ..Our findings define a previously unidentified mechanism that Ca(2+) influx via the Cav3.2 T-type Ca(2+) channel regulates Sox9 expression through the calcineurin/NFAT signaling pathway during tracheal chondrogenesis. ..
  30. Du B, Cawthorn W, Su A, Doucette C, Yao Y, Hemati N, et al. The transcription factor paired-related homeobox 1 (Prrx1) inhibits adipogenesis by activating transforming growth factor-? (TGF?) signaling. J Biol Chem. 2013;288:3036-47 pubmed publisher
    ..b>Prrx1 knockdown decreases expression of Tgfb2 and Tgfb3, and inhibition of TGF? signaling during adipogenesis mimics the ..
  31. Fritz D, Stefanovic B. RNA-binding protein RBMS3 is expressed in activated hepatic stellate cells and liver fibrosis and increases expression of transcription factor Prx1. J Mol Biol. 2007;371:585-95 pubmed
    ..The activation of HSCs is driven by transcription factors and pair-related homeobox transcription factor Prx1 was identified as one of the transcription factors involved in this process, because transcription of collagen ..
  32. Charite J, McFadden D, Olson E. The bHLH transcription factor dHAND controls Sonic hedgehog expression and establishment of the zone of polarizing activity during limb development. Development. 2000;127:2461-70 pubmed
    ..Together, these findings identify dHAND as an upstream activator of Shh expression and important transcriptional regulator of limb development. ..
  33. Kanzler B, Kuschert S, Liu Y, Mallo M. Hoxa-2 restricts the chondrogenic domain and inhibits bone formation during development of the branchial area. Development. 1998;125:2587-97 pubmed
    ..The implications of these results on the patterning of the branchial area are discussed. ..
  34. Cretekos C, Wang Y, Green E, Martin J, Rasweiler J, Behringer R. Regulatory divergence modifies limb length between mammals. Genes Dev. 2008;22:141-51 pubmed publisher
    ..We replaced a limb-specific transcriptional enhancer of the mouse Prx1 locus with the orthologous sequence from a bat...
  35. Yamagishi C, Yamagishi H, Maeda J, Tsuchihashi T, Ivey K, Hu T, et al. Sonic hedgehog is essential for first pharyngeal arch development. Pediatr Res. 2006;59:349-54 pubmed
    ..These results demonstrate that epithelial-mesenchymal signaling and transcriptional events coordinated by Shh, partly via Fgf8, is essential for cell survival and tissue outgrowth of the developing PA1. ..
  36. Barbosa A, Funato N, Chapman S, McKee M, Richardson J, Olson E, et al. Hand transcription factors cooperatively regulate development of the distal midline mesenchyme. Dev Biol. 2007;310:154-68 pubmed
    ..These findings demonstrate the importance of Hand transcription factors in the transcriptional circuitry of craniofacial and tooth development. ..
  37. Kern M, Witte D, Valerius M, Aronow B, Potter S. A novel murine homeobox gene isolated by a tissue specific PCR cloning strategy. Nucleic Acids Res. 1992;20:5189-95 pubmed
    ..Expression was observed in diverse mesenchymal cells in craniofacial, pericardial, primitive dermal, prevertebral, and genital structures. ..
  38. Compagnucci C, Fish J, Schwark M, Tarabykin V, Depew M. Pax6 regulates craniofacial form through its control of an essential cephalic ectodermal patterning center. Genesis. 2011;49:307-25 pubmed publisher
    ..Pax6 therefore regulates craniofacial form, at stages when CNC has just arrived in the frontonasal region, through its control of surface cephalic ectodermal competence to form an essential craniofacial patterning center. ..
  39. Uelmen P, Oka K, Sullivan M, Chang C, Chang T, Chan L. Tissue-specific expression and cholesterol regulation of acylcoenzyme A:cholesterol acyltransferase (ACAT) in mice. Molecular cloning of mouse ACAT cDNA, chromosomal localization, and regulation of ACAT in vivo and in vitro. J Biol Chem. 1995;270:26192-201 pubmed
    ..Two transfected Chinese hamster ovary cell lines that expressed the mouse ACAT transgene regained the ability to esterify cholesterol.(ABSTRACT TRUNCATED AT 250 WORDS) ..
  40. Zhang M, Hou M, Ge L, Miao C, Zhang J, Jing X, et al. Induction of peroxiredoxin 1 by hypoxia regulates heme oxygenase-1 via NF-κB in oral cancer. PLoS ONE. 2014;9:e105994 pubmed publisher
    Overexpression of peroxiredoxin 1 (Prx1) has been observed in numerous cancers including oral squamous cell carcinoma (OSCC). The precise molecular mechanism of up-regulation of Prx1 in carcinogenesis, however, is still poorly understood...
  41. Huang Z, Wang D, Ihida Stansbury K, Jones P, Martin J. Defective pulmonary vascular remodeling in Smad8 mutant mice. Hum Mol Genet. 2009;18:2791-801 pubmed publisher
    ..mutant pulmonary vasculature had upregulated Activin/Tgfbeta signaling and pathologic remodeling with aberrant Prx1 and Tenascin-C expression...
  42. Mallo M, Schrewe H, Martin J, Olson E, Ohnemus S. Assembling a functional tympanic membrane: signals from the external acoustic meatus coordinate development of the malleal manubrium. Development. 2000;127:4127-36 pubmed
    ..Finally, we show that the EAM is the source of two quite opposite activities, one that induces chondrogenesis and another that represses it. The combination of these two activities results in the proper positioning of the manubrium. ..
  43. Vallejo Illarramendi A, Zang K, Reichardt L. Focal adhesion kinase is required for neural crest cell morphogenesis during mouse cardiovascular development. J Clin Invest. 2009;119:2218-30 pubmed publisher
    ..Our results indicate that FAK plays an essential role in cardiac outflow tract development by promoting the activation of molecules such as Crkl and Erk1/2...
  44. Fekete D. Development of the vertebrate ear: insights from knockouts and mutants. Trends Neurosci. 1999;22:263-9 pubmed
    ..Genes that are regionally expressed in the inner ear can, when absent or mutated, yield selective ablation of specific inner-ear structures or cell types. ..
  45. Norris R, Scott K, Moore C, Stetten G, Brown C, Jabs E, et al. Human PRRX1 and PRRX2 genes: cloning, expression, genomic localization, and exclusion as disease genes for Nager syndrome. Mamm Genome. 2000;11:1000-5 pubmed
    In this study, we extend our examination of the function of the Prrx1 (a.k.a Mhox, Prx1, K-2, and Pmx1) as well as Prrx2 (a.k.a...
  46. Akiyama R, Kawakami H, Taketo M, Evans S, Wada N, Petryk A, et al. Distinct populations within Isl1 lineages contribute to appendicular and facial skeletogenesis through the ?-catenin pathway. Dev Biol. 2014;387:37-48 pubmed publisher
  47. Hirota J, Mombaerts P. The LIM-homeodomain protein Lhx2 is required for complete development of mouse olfactory sensory neurons. Proc Natl Acad Sci U S A. 2004;101:8751-5 pubmed
    ..OSN development appears to be arrested between the terminal differentiation into neurons and the transition to immature neurons. Thus, Lhx2 is required for complete development of OSNs in mice. ..
  48. Cserjesi P, Lilly B, Bryson L, Wang Y, Sassoon D, Olson E. MHox: a mesodermally restricted homeodomain protein that binds an essential site in the muscle creatine kinase enhancer. Development. 1992;115:1087-101 pubmed
    ..Here we describe a novel homeodomain protein, MHox, that binds an A+T-rich element in the muscle creatine kinase (MCK) enhancer that is essential for muscle-specific ..
  49. Weiss K, Ruddle F, Bollekens J. Dlx and other homeobox genes in the morphological development of the dentition. Connect Tissue Res. 1995;32:35-40 pubmed
    ..Dlx1, and Dlx2, and identified several other homeobox genes not previously known to be expressed in teeth: Dbx, MHox, and Mox2A, plus an a additional Dlx gene, Dlx7...
  50. Lu X, Beck G, Gilbert L, Camalier C, Bateman N, Hood B, et al. Identification of the homeobox protein Prx1 (MHox, Prrx-1) as a regulator of osterix expression and mediator of tumor necrosis factor ? action in osteoblast differentiation. J Bone Miner Res. 2011;26:209-19 pubmed publisher
    ..One protein, the paired related homeobox protein (Prx1), had been shown previously to have a critical role in limb bud formation and skeletal patterning...
  51. Canalis E, Zanotti S, Beamer W, Economides A, Smerdel Ramoya A. Connective tissue growth factor is required for skeletal development and postnatal skeletal homeostasis in male mice. Endocrinology. 2010;151:3490-501 pubmed publisher lox sequences with mice expressing the Cre recombinase under the control of the paired-related homeobox gene 1 (Prx1) enhancer (Prx1-Cre) or the osteocalcin promoter (Oc-Cre)...
  52. Jiang F, Stefanovic B. Homeobox gene Prx1 is expressed in activated hepatic stellate cells and transactivates collagen alpha1(I) promoter. Exp Biol Med (Maywood). 2008;233:286-96 pubmed publisher
    ..Upon fibrogenic stimulus, HSCs become activated and increase synthesis of type I collagen 50-100 fold. Prx1 and Prx2 are two homeobox transcription factors which are required for mesenchymal tissue formation during ..
  53. Sylva M, Li V, Buffing A, van Es J, van den Born M, van der Velden S, et al. The BMP antagonist follistatin-like 1 is required for skeletal and lung organogenesis. PLoS ONE. 2011;6:e22616 pubmed publisher
    ..Taken together, these observations show that Fstl1 is a crucial regulator in BMP signalling during mouse development. ..