Gene Symbol: Pax3
Description: paired box 3
Alias: Pax-3, Splchl2, splotch, paired box protein Pax-3, PAX3/FKHR fusion, paired box gene 3
Species: mouse
Products:     Pax3

Top Publications

  1. Gross M, Dottori M, Goulding M. Lbx1 specifies somatosensory association interneurons in the dorsal spinal cord. Neuron. 2002;34:535-49 pubmed
    ..Lbx1, therefore, plays a critical role in the development of sensory pathways in the spinal cord that relay pain and touch. ..
  2. Lagha M, Brunelli S, Messina G, Cumano A, Kume T, Relaix F, et al. Pax3:Foxc2 reciprocal repression in the somite modulates muscular versus vascular cell fate choice in multipotent progenitors. Dev Cell. 2009;17:892-9 pubmed publisher different lineages such as vascular smooth and skeletal muscle, regulated by the transcription factors Foxc2 and Pax3, respectively. Here we show reciprocal inhibition between Pax3 and Foxc2 in the mouse embryo...
  3. Seo K. Dmrt2 and Pax3 double-knockout mice show severe defects in embryonic myogenesis. Comp Med. 2007;57:460-8 pubmed
    ..Several transcription factors from the dermomyotome affect embryonic myogenesis. Among these, Dmrt2 and Pax3 were tested for genetic and functional interactions during embryonic myogenesis by evaluating myogenin and desmin ..
  4. Relaix F, Rocancourt D, Mansouri A, Buckingham M. A Pax3/Pax7-dependent population of skeletal muscle progenitor cells. Nature. 2005;435:948-53 pubmed
    ..We have identified a new cell population that expresses the transcription factors Pax3 and Pax7 (paired box proteins 3 and 7) but no skeletal-muscle-specific markers...
  5. Kioussi C, Briata P, Baek S, Rose D, Hamblet N, Herman T, et al. Identification of a Wnt/Dvl/beta-Catenin --> Pitx2 pathway mediating cell-type-specific proliferation during development. Cell. 2002;111:673-85 pubmed
  6. Gong C, Kim Y, Woeller C, Tang Y, Maquat L. SMD and NMD are competitive pathways that contribute to myogenesis: effects on PAX3 and myogenin mRNAs. Genes Dev. 2009;23:54-66 pubmed publisher
    ..We discuss the remarkable balance between SMD and the two types of NMD in view of data indicating that PAX3 mRNA is an SMD target whose decay promotes myogenesis whereas myogenin mRNA is a classical NMD target encoding a ..
  7. L honoré A, Coulon V, Marcil A, Lebel M, Lafrance Vanasse J, Gage P, et al. Sequential expression and redundancy of Pitx2 and Pitx3 genes during muscle development. Dev Biol. 2007;307:421-33 pubmed
    ..factors acting during muscle development, including bHLH muscle regulatory factors (MRFs), the paired factors Pax3 and Pax7 and the homeobox factors Six1 and Six4...
  8. Schäfer K, Neuhaus P, Kruse J, Braun T. The homeobox gene Lbx1 specifies a subpopulation of cardiac neural crest necessary for normal heart development. Circ Res. 2003;92:73-80 pubmed
    ..activity of the Lbx1 promoter, as indicated by a LacZ reporter gene, is upregulated in the hearts of Lbx1(+/-):splotch(1H)/splotch(1H) and Lbx1(-/-) mice, indicating that Pax3 and Lbx1 participate in a negative regulatory feedback ..
  9. Marshall A, van der Ent M, Grosveld G. PAX3-FOXO1 and FGFR4 in alveolar rhabdomyosarcoma. Mol Carcinog. 2012;51:807-15 pubmed publisher
    ..FGFR4 as a direct transcriptional target of the alveolar rhabdomyosarcoma (ARMS) specific fusion protein, PAX3-FOXO1...

More Information


  1. Stamataki D, Kastrinaki M, Mankoo B, Pachnis V, Karagogeos D. Homeodomain proteins Mox1 and Mox2 associate with Pax1 and Pax3 transcription factors. FEBS Lett. 2001;499:274-8 pubmed
    ..Pax1 and Pax3 gene products are also implicated in these processes...
  2. Engleka K, Gitler A, Zhang M, Zhou D, High F, Epstein J. Insertion of Cre into the Pax3 locus creates a new allele of Splotch and identifies unexpected Pax3 derivatives. Dev Biol. 2005;280:396-406 pubmed
    ..Pax3-deficient Splotch embryos display neural tube and neural crest defects and lack hypaxial muscles...
  3. Lagutina I, Conway S, Sublett J, Grosveld G. Pax3-FKHR knock-in mice show developmental aberrations but do not develop tumors. Mol Cell Biol. 2002;22:7204-16 pubmed
    ..These translocations result in the formation of the PAX3-FKHR and PAX7-FKHR fusion genes, which are thought to play a causal role in the genesis of this disease...
  4. Vinagre T, Moncaut N, Carapuco M, Novoa A, Bom J, Mallo M. Evidence for a myotomal Hox/Myf cascade governing nonautonomous control of rib specification within global vertebral domains. Dev Cell. 2010;18:655-61 pubmed publisher
    ..Our findings offer a new perspective of how Hox genes produce global patterns in the axial skeleton and support a redundant nonmyogenic role of Myf5 and Myf6 in rib formation. ..
  5. Haldar M, Hancock J, Coffin C, Lessnick S, Capecchi M. A conditional mouse model of synovial sarcoma: insights into a myogenic origin. Cancer Cell. 2007;11:375-88 pubmed
    ..We also show that early widespread expression of the fusion protein disrupts normal embryogenesis, causing lethality. ..
  6. Bajard L, Relaix F, Lagha M, Rocancourt D, Daubas P, Buckingham M. A novel genetic hierarchy functions during hypaxial myogenesis: Pax3 directly activates Myf5 in muscle progenitor cells in the limb. Genes Dev. 2006;20:2450-64 pubmed
    ..Null mutations in Pax3, a key regulator of skeletal muscle formation, lead to cell death in this domain...
  7. Kassar Duchossoy L, Giacone E, Gayraud Morel B, Jory A, Gomès D, Tajbakhsh S. Pax3/Pax7 mark a novel population of primitive myogenic cells during development. Genes Dev. 2005;19:1426-31 pubmed
    ..We identify a novel population of resident Pax3+/Pax7+, muscle marker-negative cells throughout development...
  8. Crist C, Montarras D, Pallafacchina G, Rocancourt D, Cumano A, Conway S, et al. Muscle stem cell behavior is modified by microRNA-27 regulation of Pax3 expression. Proc Natl Acad Sci U S A. 2009;106:13383-7 pubmed publisher
    Skeletal muscle stem cells are regulated by Pax3/7...
  9. Gross M, Moran Rivard L, Velasquez T, Nakatsu M, Jagla K, Goulding M. Lbx1 is required for muscle precursor migration along a lateral pathway into the limb. Development. 2000;127:413-24 pubmed
    ..Previous studies have shown that Pax3, together with the c-Met receptor tyrosine kinase and its ligand Scatter Factor (SF) are necessary for the ..
  10. Cheng H, Kim M, Valerius M, Surendran K, Schuster Gossler K, Gossler A, et al. Notch2, but not Notch1, is required for proximal fate acquisition in the mammalian nephron. Development. 2007;134:801-11 pubmed
    ..These results establish distinct (non-redundant), instructive roles for Notch receptors in nephron segmentation. ..
  11. Mansouri A, Pla P, Larue L, Gruss P. Pax3 acts cell autonomously in the neural tube and somites by controlling cell surface properties. Development. 2001;128:1995-2005 pubmed
    ..Several loss-of-function mutations are correlated with the Splotch phenotype in mice and Waardenburg syndrome in humans...
  12. Dietrich S, Abou Rebyeh F, Brohmann H, Bladt F, Sonnenberg Riethmacher E, Yamaai T, et al. The role of SF/HGF and c-Met in the development of skeletal muscle. Development. 1999;126:1621-9 pubmed
    ..They lack muscle groups that derive from migratory precursor cells, but display otherwise normal skeletal musculature. ..
  13. Abraham J, Núñez Álvarez Y, Hettmer S, Carrió E, Chen H, Nishijo K, et al. Lineage of origin in rhabdomyosarcoma informs pharmacological response. Genes Dev. 2014;28:1578-91 pubmed publisher
    ..We used conditional genetic mouse models of aRMS to activate the pathognomonic Pax3:Foxo1 fusion oncogene and inactivate p53 in several stages of prenatal and postnatal muscle development...
  14. Chen L, Guo Q, Li J. Transcription factor Gbx2 acts cell-nonautonomously to regulate the formation of lineage-restriction boundaries of the thalamus. Development. 2009;136:1317-26 pubmed publisher
    ..We propose that, within the developing thalamus, the dynamic and differential expression of Gbx2 may be involved in the specific segregation of thalamic neurons, leading to partition of the thalamus into different nuclei. ..
  15. Abou Khalil R, Le Grand F, Pallafacchina G, Valable S, Authier F, Rudnicki M, et al. Autocrine and paracrine angiopoietin 1/Tie-2 signaling promotes muscle satellite cell self-renewal. Cell Stem Cell. 2009;5:298-309 pubmed publisher
    ..We propose that Ang1/Tie-2 signaling regulates mpc self-renewal by controlling the return to quiescence of a subset of satellite cells...
  16. Havis E, Coumailleau P, Bonnet A, Bismuth K, Bonnin M, Johnson R, et al. Sim2 prevents entry into the myogenic program by repressing MyoD transcription during limb embryonic myogenesis. Development. 2012;139:1910-20 pubmed publisher
    ..This MyoD repression is predominant in ventral limb regions and is likely to contribute to the differential increase of the global mass of ventral muscles versus dorsal muscles. ..
  17. Pritchard C, Grosveld G, Hollenbach A. Alternative splicing of Pax3 produces a transcriptionally inactive protein. Gene. 2003;305:61-9 pubmed
    b>Pax3, a member of the paired-class homeodomain family of transcription factors, plays an important role in embryonic development of neurepithelium and mesenchyme-derived tissues in the mouse and is an early marker for myogenic ..
  18. Pruitt S, Bussman A, Maslov A, Natoli T, Heinaman R. Hox/Pbx and Brn binding sites mediate Pax3 expression in vitro and in vivo. Gene Expr Patterns. 2004;4:671-85 pubmed
    b>Pax3 is a paired-homeodomain class transcription factor that serves a role in dorsal-ventral and medial-lateral patterning during vertebrate embryogenesis...
  19. Lang D, Lu M, Huang L, Engleka K, Zhang M, Chu E, et al. Pax3 functions at a nodal point in melanocyte stem cell differentiation. Nature. 2005;433:884-7 pubmed
    ..Here we describe the molecular details of a nodal point in adult melanocyte stem cell differentiation in which Pax3 simultaneously functions to initiate a melanogenic cascade while acting downstream to prevent terminal ..
  20. Lepper C, Conway S, Fan C. Adult satellite cells and embryonic muscle progenitors have distinct genetic requirements. Nature. 2009;460:627-31 pubmed publisher
    Myogenic potential, survival and expansion of mammalian muscle progenitors depend on the myogenic determinants Pax3 and Pax7 embryonically, and Pax7 alone perinatally...
  21. Hutcheson D, Zhao J, Merrell A, Haldar M, Kardon G. Embryonic and fetal limb myogenic cells are derived from developmentally distinct progenitors and have different requirements for beta-catenin. Genes Dev. 2009;23:997-1013 pubmed publisher
    ..b>Pax3 and Pax7 are expressed by somitic myogenic progenitors and are critical myogenic determinants...
  22. Mayanil C, Pool A, Nakazaki H, Reddy A, Mania Farnell B, Yun B, et al. Regulation of murine TGFbeta2 by Pax3 during early embryonic development. J Biol Chem. 2006;281:24544-52 pubmed
    Previously our laboratory identified TGFbeta2 as a potential downstream target of Pax3 by utilizing microarray analysis and promoter data base mining (Mayanil, C. S. K., George, D., Freilich, L., Miljan, E. J., Mania-Farnell, B. J...
  23. Fenby B, Fotaki V, Mason J. Pax3 regulates Wnt1 expression via a conserved binding site in the 5' proximal promoter. Biochim Biophys Acta. 2008;1779:115-21 pubmed
    ..Here, we demonstrate a direct interaction between two such factors, the paired-type transcription factor Pax3 and the secretory glycoprotein Wnt1...
  24. Alder O, Lavial F, Helness A, Brookes E, Pinho S, Chandrashekran A, et al. Ring1B and Suv39h1 delineate distinct chromatin states at bivalent genes during early mouse lineage commitment. Development. 2010;137:2483-92 pubmed publisher
  25. Choi K, Harfe B. Hedgehog signaling is required for formation of the notochord sheath and patterning of nuclei pulposi within the intervertebral discs. Proc Natl Acad Sci U S A. 2011;108:9484-9 pubmed publisher
    ..As notochord cells form nuclei pulposi, we propose that the notochord sheath functions as a "wrapper" around the notochord to constrain these cells along the vertebral column. ..
  26. Keller C, Arenkiel B, Coffin C, El Bardeesy N, Depinho R, Capecchi M. Alveolar rhabdomyosarcomas in conditional Pax3:Fkhr mice: cooperativity of Ink4a/ARF and Trp53 loss of function. Genes Dev. 2004;18:2614-26 pubmed
    ..We report the first mouse model of alveolar rhabdomyosarcoma using a conditional Pax3:Fkhr knock-in allele whose activation in late embryogenesis and postnatally is targeted to terminally ..
  27. Bussen M, Petry M, Schuster Gossler K, Leitges M, Gossler A, Kispert A. The T-box transcription factor Tbx18 maintains the separation of anterior and posterior somite compartments. Genes Dev. 2004;18:1209-21 pubmed
    ..In summary, Tbx18 appears to act downstream of Mesp2 and Delta/Notch signaling to maintain the separation of anterior and posterior somite compartments. ..
  28. Relaix F, Rocancourt D, Mansouri A, Buckingham M. Divergent functions of murine Pax3 and Pax7 in limb muscle development. Genes Dev. 2004;18:1088-105 pubmed
    Pax genes encode evolutionarily conserved transcription factors that play critical roles in development. Pax3 and Pax7 constitute one of the four Pax subfamilies...
  29. Fedtsova N, Perris R, Turner E. Sonic hedgehog regulates the position of the trigeminal ganglia. Dev Biol. 2003;261:456-69 pubmed
    ..The ectopic ganglia originate from Pax3-expressing regions of the surface ectoderm that normally contribute to the ophthalmic trigeminal (op5), and can ..
  30. Bröhl D, Vasyutina E, Czajkowski M, Griger J, Rassek C, Rahn H, et al. Colonization of the satellite cell niche by skeletal muscle progenitor cells depends on Notch signals. Dev Cell. 2012;23:469-81 pubmed publisher
    ..We conclude that Notch signals control homing of satellite cells, stimulating them to contribute to their own microenvironment and to adhere to myofibers. ..
  31. Zhao T, Gan Q, Stokes A, Lassiter R, Wang Y, Chan J, et al. ?-catenin regulates Pax3 and Cdx2 for caudal neural tube closure and elongation. Development. 2014;141:148-57 pubmed publisher
    ..of ?-catenin in the dorsal neural folds of mouse embryos represses the expression of the homeobox-containing genes Pax3 and Cdx2 at the dorsal posterior neuropore (PNP), and subsequently diminishes the expression of the Wnt/?-catenin ..
  32. Seo K, Wang Y, Kokubo H, Kettlewell J, Zarkower D, Johnson R. Targeted disruption of the DM domain containing transcription factor Dmrt2 reveals an essential role in somite patterning. Dev Biol. 2006;290:200-10 pubmed
    ..defects are alterations in the expression patterns of dermomyotomal and myotomal transcription factors including Pax3, Paraxis, Myf5, myogenin, Mrf4 and MyoD. Despite these defects, embryos harvested from E13...
  33. Relaix F, Polimeni M, Rocancourt D, Ponzetto C, Schafer B, Buckingham M. The transcriptional activator PAX3-FKHR rescues the defects of Pax3 mutant mice but induces a myogenic gain-of-function phenotype with ligand-independent activation of Met signaling in vivo. Genes Dev. 2003;17:2950-65 pubmed
    b>Pax3 is a key transcription factor implicated in development and human disease...
  34. Barber T, Barber M, Tomescu O, Barr F, Ruben S, Friedman T. Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcoma. Genomics. 2002;79:278-84 pubmed
    b>PAX3 is a transcription factor important for neural, muscle, and facial development in vertebrates...
  35. Miller P, Dietz K, Hollenbach A. Identification of serine 205 as a site of phosphorylation on Pax3 in proliferating but not differentiating primary myoblasts. Protein Sci. 2008;17:1979-86 pubmed publisher
    b>Pax3, a member of the paired class homeodomain family of transcription factors, is essential for early skeletal muscle development. Previously, others and we have shown that the stability of Pax3 is regulated on a post-translational level...
  36. Hu P, Geles K, Paik J, Depinho R, Tjian R. Codependent activators direct myoblast-specific MyoD transcription. Dev Cell. 2008;15:534-46 pubmed publisher
    ..Here, we identify MyoD, a key regulator of myogenesis, as a direct target of FoxO3 and Pax3/7 in myoblasts...
  37. Vasyutina E, Lenhard D, Wende H, Erdmann B, Epstein J, Birchmeier C. RBP-J (Rbpsuh) is essential to maintain muscle progenitor cells and to generate satellite cells. Proc Natl Acad Sci U S A. 2007;104:4443-8 pubmed
    ..In addition, satellite cells are not formed late in fetal development in conditional RBP-J mutant mice. We conclude that RBP-J is required in the developing muscle to set aside proliferating progenitors and satellite cells...
  38. Blitz E, Viukov S, Sharir A, Shwartz Y, Galloway J, Pryce B, et al. Bone ridge patterning during musculoskeletal assembly is mediated through SCX regulation of Bmp4 at the tendon-skeleton junction. Dev Cell. 2009;17:861-73 pubmed publisher
    ..This study establishes a mechanistic basis for tendon-skeleton regulatory interactions during musculoskeletal assembly and bone secondary patterning. ..
  39. Relaix F, Montarras D, Zaffran S, Gayraud Morel B, Rocancourt D, Tajbakhsh S, et al. Pax3 and Pax7 have distinct and overlapping functions in adult muscle progenitor cells. J Cell Biol. 2006;172:91-102 pubmed
    ..We show that Pax3, the paralogue of Pax7, is also present in both quiescent and activated satellite cells in many skeletal muscles...
  40. Lang D, Epstein J. Sox10 and Pax3 physically interact to mediate activation of a conserved c-RET enhancer. Hum Mol Genet. 2003;12:937-45 pubmed
    ..One example is Waardenburg syndrome that can be caused by mutations in the PAX3, SOX10 or MITF genes...
  41. Schafer K, Braun T. Early specification of limb muscle precursor cells by the homeobox gene Lbx1h. Nat Genet. 1999;23:213-6 pubmed
    ..6), Tbx1 (ref. 7), Mox2 (ref. 8), Six1 and Six2 (ref. 9), Pitx2, Pax3 (refs 10,11) and Lbx1h (refs 12,13), are expressed in migrating limb precursor cells...
  42. Barber T, Barber M, Cloutier T, Friedman T. PAX3 gene structure, alternative splicing and evolution. Gene. 1999;237:311-9 pubmed
    ..syndrome (types 1 and 3), Craniofacial-deafness-hand syndrome and alveolar rhabdomyosarcoma in humans and the Splotch phenotype in mice...
  43. Collins C, Gnocchi V, White R, Boldrin L, Perez Ruiz A, Relaix F, et al. Integrated functions of Pax3 and Pax7 in the regulation of proliferation, cell size and myogenic differentiation. PLoS ONE. 2009;4:e4475 pubmed publisher
    b>Pax3 and Pax7 are paired-box transcription factors with roles in developmental and adult regenerative myogenesis...
  44. Dude C, Kuan C, Bradshaw J, Greene N, Relaix F, Stark M, et al. Activation of Pax3 target genes is necessary but not sufficient for neurogenesis in the ophthalmic trigeminal placode. Dev Biol. 2009;326:314-26 pubmed publisher
    ..Pax3 can act as a transcriptional activator or repressor, depending on the cellular context. We show using mouse Splotch(2H) mutants that Pax3 is necessary for the normal neuronal differentiation of opV placode cells...
  45. Borycki A, Li J, Jin F, Emerson C, Epstein J. Pax3 functions in cell survival and in pax7 regulation. Development. 1999;126:1665-74 pubmed
    ..and gene expression in presomitic mesoderm, somites and neural tube of developing wild-type and Pax3 mutant (Splotch) mouse embryos...
  46. Darabi R, Gehlbach K, Bachoo R, Kamath S, Osawa M, Kamm K, et al. Functional skeletal muscle regeneration from differentiating embryonic stem cells. Nat Med. 2008;14:134-43 pubmed publisher
    ..Here we show that expression of the transcription factor Pax3 during embryoid body differentiation enhances both paraxial mesoderm formation and the myogenic potential of the ..
  47. Underwood T, Amin J, Lillycrop K, Blaydes J. Dissection of the functional interaction between p53 and the embryonic proto-oncoprotein PAX3. FEBS Lett. 2007;581:5831-5 pubmed
    ..and cancer cells derived from human melanomas have identified a critical role for the transcription factor PAX3 in the suppression of p53 protein accumulation and p53-dependent apoptosis...
  48. Buckiova D, Syka J. Development of the inner ear in Splotch mutant mice. Neuroreport. 2004;15:2001-5 pubmed
    The Splotch mouse, a Pax 3 mutation, represents a model of Waardenburg syndrome I...
  49. Wiggan O, Fadel M, Hamel P. Pax3 induces cell aggregation and regulates phenotypic mesenchymal-epithelial interconversion. J Cell Sci. 2002;115:517-29 pubmed
    ..Although mutations to Pax3 and other Pax-family genes in both mice and humans result in numerous tissue-specific morphological defects, little ..
  50. Olaopa M, Zhou H, Snider P, Wang J, Schwartz R, Moon A, et al. Pax3 is essential for normal cardiac neural crest morphogenesis but is not required during migration nor outflow tract septation. Dev Biol. 2011;356:308-22 pubmed publisher
    Systemic loss-of-function studies have demonstrated that Pax3 transcription factor expression is essential for dorsal neural tube, early neural crest and muscle cell lineage morphogenesis...
  51. Lagha M, Sato T, Regnault B, Cumano A, Zuniga A, Licht J, et al. Transcriptome analyses based on genetic screens for Pax3 myogenic targets in the mouse embryo. BMC Genomics. 2010;11:696 pubmed publisher
    b>Pax3 is a key upstream regulator of the onset of myogenesis, controlling progenitor cell survival and behaviour as well as entry into the myogenic programme...
  52. Sharir A, Stern T, Rot C, Shahar R, Zelzer E. Muscle force regulates bone shaping for optimal load-bearing capacity during embryogenesis. Development. 2011;138:3247-59 pubmed publisher
    ..Our findings invoke a common mechanism that permits the formation of different circumferential outlines in different bones. ..
  53. Mankoo B, Collins N, Ashby P, Grigorieva E, Pevny L, Candia A, et al. Mox2 is a component of the genetic hierarchy controlling limb muscle development. Nature. 1999;400:69-73 pubmed
    ..encoding the basic helix-loop-helix (bHLH) myogenic regulatory factors (MRFs; MyoD, Myf5, myogenin and MRF4) and Pax3, a paired-type homeobox gene that is critical for the development of limb musculature...
  54. Li J, Liu K, Jin F, Lu M, Epstein J. Transgenic rescue of congenital heart disease and spina bifida in Splotch mice. Development. 1999;126:2495-503 pubmed
    Pax3-deficient Splotch mice display neural tube defects and an array of neural crest related abnormalities including defects in the cardiac outflow tract, dorsal root ganglia and pigmentation...
  55. Vasyutina E, Stebler J, Brand Saberi B, Schulz S, Raz E, Birchmeier C. CXCR4 and Gab1 cooperate to control the development of migrating muscle progenitor cells. Genes Dev. 2005;19:2187-98 pubmed
    ..Our analysis reveals a role of SDF1/CXCR4 signaling in the development of migrating muscle progenitors and shows that a threshold number of progenitor cells is required to generate muscle of appropriate size. ..
  56. Freyer L, Aggarwal V, Morrow B. Dual embryonic origin of the mammalian otic vesicle forming the inner ear. Development. 2011;138:5403-14 pubmed publisher
    ..Using Wnt1-Cre, Pax3(Cre/+) and Hoxb1(Cre/+) mice to label and fate map cranial NEC lineages, we have demonstrated that cells from the ..
  57. Keller C, Hansen M, Coffin C, Capecchi M. Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: implications for alveolar rhabdomyosarcoma cell of origin. Genes Dev. 2004;18:2608-13 pubmed
    To investigate the role of the translocation-associated gene Pax3:Fkhr in alveolar rhabdomyosarcomas, we generated a Cre-mediated conditional knock-in of Pax3:Fkhr into the mouse Pax3 locus...
  58. Abraham J, Chua Y, Glover J, Tyner J, Loriaux M, Kilcoyne A, et al. An adaptive Src-PDGFRA-Raf axis in rhabdomyosarcoma. Biochem Biophys Res Commun. 2012;426:363-8 pubmed publisher
    ..These results suggest that an adaptive Src-Pdgfra-Raf-Mapk axis is relevant to PDGFRA inhibition in rhabdomyosarcoma. ..
  59. Boutet S, Disatnik M, Chan L, Iori K, Rando T. Regulation of Pax3 by proteasomal degradation of monoubiquitinated protein in skeletal muscle progenitors. Cell. 2007;130:349-62 pubmed
    b>Pax3 and Pax7 play distinct but overlapping roles in developmental and postnatal myogenesis. The mechanisms involved in the differential regulation of these highly homologous proteins are unknown...
  60. Coumailleau P, Duprez D. Sim1 and Sim2 expression during chick and mouse limb development. Int J Dev Biol. 2009;53:149-57 pubmed publisher
    ..In addition, chick and mouse Sim2 gene expression is enhanced in limb ventral muscle masses versus dorsal ventral muscle masses. Our results provide a basis for further functional analysis of the Sim genes in limb muscle formation. ..
  61. Gensch N, Borchardt T, Schneider A, Riethmacher D, Braun T. Different autonomous myogenic cell populations revealed by ablation of Myf5-expressing cells during mouse embryogenesis. Development. 2008;135:1597-604 pubmed publisher
    ..Individual myogenic cell lineages seem to substitute for each other within the developing embryo. ..
  62. Li G, Kikuchi K, Radka M, Abraham J, Rubin B, Keller C. IL-4 receptor blockade abrogates satellite cell: rhabdomyosarcoma fusion and prevents tumor establishment. Stem Cells. 2013;31:2304-12 pubmed publisher
    ..Taken together, this study supports a potential role of tumor cell-host cell fusion and the strong therapeutic potential of IL-4R blockade to prevent the establishment of RMS tumors at new anatomical sites. ..
  63. Prunotto C, Crepaldi T, Forni P, Ieraci A, Kelly R, Tajbakhsh S, et al. Analysis of Mlc-lacZ Met mutants highlights the essential function of Met for migratory precursors of hypaxial muscles and reveals a role for Met in the development of hyoid arch-derived facial muscles. Dev Dyn. 2004;231:582-91 pubmed
    The Pax3 and c-met genes are necessary for the development of tongue, diaphragm, and limb muscles...
  64. Chang C, Stankunas K, Shang C, Kao S, Twu K, Cleary M. Pbx1 functions in distinct regulatory networks to pattern the great arteries and cardiac outflow tract. Development. 2008;135:3577-86 pubmed publisher
    ..Pbx1 deficiency also results in the failure of cardiac OFT septation. Pbx1-null embryos lose a transient burst of Pax3 expression in premigratory cardiac neural crest cells (NCCs) that ultimately specifies cardiac NCC function for OFT ..
  65. Theis S, Patel K, Valasek P, Otto A, Pu Q, Harel I, et al. The occipital lateral plate mesoderm is a novel source for vertebrate neck musculature. Development. 2010;137:2961-71 pubmed publisher
    ..In concordance with this conclusion, their connective tissue is neural crest in origin. Finally, we provide evidence that the mechanism by which these neck muscles develop is conserved in vertebrates...
  66. Haase G, Dessaud E, Garces A, de Bovis B, Birling M, Filippi P, et al. GDNF acts through PEA3 to regulate cell body positioning and muscle innervation of specific motor neuron pools. Neuron. 2002;35:893-905 pubmed
    ..Thus, GDNF acts as a peripheral signal to induce PEA3 expression in specific motor neuron pools thereby regulating both cell body position and muscle innervation. ..
  67. Sato T, Rocancourt D, Marques L, Thorsteinsdóttir S, Buckingham M. A Pax3/Dmrt2/Myf5 regulatory cascade functions at the onset of myogenesis. PLoS Genet. 2010;6:e1000897 pubmed publisher
    ..These multipotent stem cells express Pax3, and this expression is maintained in the myogenic lineage where Pax3 plays an important role...
  68. Pani L, Horal M, Loeken M. Rescue of neural tube defects in Pax-3-deficient embryos by p53 loss of function: implications for Pax-3- dependent development and tumorigenesis. Genes Dev. 2002;16:676-80 pubmed
    ..We previously showed that apoptosis is associated with neural tube defects (NTDs) in Pax-3-deficient Splotch (Sp/Sp) embryos...
  69. Shih H, Gross M, Kioussi C. Expression pattern of the homeodomain transcription factor Pitx2 during muscle development. Gene Expr Patterns. 2007;7:441-51 pubmed
    ..5 demonstrated that Pitx2 neatly marked the areas of muscle anlagen, that Pax3, Lbx1, and the muscle regulatory factors (MRFs) stained only subsets of Pitx2(+) cells within these areas, and that ..
  70. Kumar D, Shadrach J, Wagers A, Lassar A. Id3 is a direct transcriptional target of Pax7 in quiescent satellite cells. Mol Biol Cell. 2009;20:3170-7 pubmed publisher
    Pax7 is a key regulator of skeletal muscle stem cells and is required along with Pax3 to generate skeletal muscle precursors. We have identified a collection of genes induced by either Pax3 or Pax7 in C2C12 muscle cells...
  71. Bendall A, Ding J, Hu G, Shen M, Abate Shen C. Msx1 antagonizes the myogenic activity of Pax3 in migrating limb muscle precursors. Development. 1999;126:4965-76 pubmed
    ..Here we show that in chicken embryos, expression of the Msx1 homeobox gene overlaps with Pax3 in migrating limb muscle precursors, which are committed myoblasts that do not express myogenic differentiation ..
  72. Lagha M, Kormish J, Rocancourt D, Manceau M, Epstein J, Zaret K, et al. Pax3 regulation of FGF signaling affects the progression of embryonic progenitor cells into the myogenic program. Genes Dev. 2008;22:1828-37 pubmed publisher
    b>Pax3/7-dependent stem cells play an essential role in skeletal muscle development. We now show that Fgfr4 lies genetically downstream from Pax3 and is a direct target...
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