Six1

Summary

Gene Symbol: Six1
Description: sine oculis-related homeobox 1
Alias: BB138287, homeobox protein SIX1, sine oculis homeobox homolog 1, sine oculis-related homeobox 1 homolog
Species: mouse

Top Publications

  1. doi Initiation of olfactory placode development and neurogenesis is blocked in mice lacking both Six1 and Six4
    Binglai Chen
    Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine of NYU, New York, NY 10029, USA
    Dev Biol 326:75-85. 2009
  2. ncbi Mutation in the transcriptional coactivator EYA4 causes dilated cardiomyopathy and sensorineural hearing loss
    Jost Schönberger
    Harvard Medical School, Department of Genetics, 77 Avenue Louis Pasteur, Boston, Massachusetts 02115, USA
    Nat Genet 37:418-22. 2005
  3. ncbi Expression profiling identifies the cytoskeletal organizer ezrin and the developmental homeoprotein Six-1 as key metastatic regulators
    Yanlin Yu
    Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Nat Med 10:175-81. 2004
  4. pmc The Six1 homeoprotein stimulates tumorigenesis by reactivation of cyclin A1
    Ricardo D Coletta
    Department of Obstetrics and Gynecology, University Colorado Health Sciences Center, Denver, CO 80262, USA
    Proc Natl Acad Sci U S A 101:6478-83. 2004
  5. pmc Expression of Six1 homeobox gene during development of the mouse submandibular salivary gland
    E L McCoy
    Program in Molecular Biology, University of Colorado School of Medicine, Denver, CO, USA
    Oral Dis 15:407-13. 2009
  6. pmc Six1 and Eya1 are critical regulators of peri-cloacal mesenchymal progenitors during genitourinary tract development
    Chen Wang
    Department of Urology, Children s Hospital Boston, 300 Longwood Avenue, Harvard Medical School, Boston, MA 02115, USA
    Dev Biol 360:186-94. 2011
  7. pmc Eya4-deficient mice are a model for heritable otitis media
    Frederic F S Depreux
    Department of Genetics, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Clin Invest 118:651-8. 2008
  8. pmc Eya1 and Six1 are essential for early steps of sensory neurogenesis in mammalian cranial placodes
    Dan Zou
    McLaughlin Research Institute for Biomedical Sciences, 1520 23rd Street South, Great Falls, MT 59405, USA
    Development 131:5561-72. 2004
  9. pmc Six1 regulates stem cell repair potential and self-renewal during skeletal muscle regeneration
    Fabien Le Grand
    Institut National de la Santé et de la Recherche Médicale U1016, Institut Cochin, Paris 75014, France
    J Cell Biol 198:815-32. 2012
  10. pmc Discovery, optimization and validation of an optimal DNA-binding sequence for the Six1 homeodomain transcription factor
    Yubing Liu
    Ottawa Institute of Systems Biology and Biochemistry, Microbiology and Immunology Department, Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada
    Nucleic Acids Res 40:8227-39. 2012

Research Grants

Scientific Experts

  • Yuko Suzuki
  • Pin Xian Xu
  • K Kawakami
  • Wangjun Wu
  • Bradley S Gordon
  • Xue Li
  • Li Chai
  • Yongsu Jeong
  • Shigeru Sato
  • Ahmed H K El-Hashash
  • Kenneth A Walker
  • Pascal Maire
  • Keiko Ikeda
  • Raphaelle Grifone
  • Josiane Demignon
  • Dan Zou
  • Yubing Liu
  • Christine Laclef
  • Derek Silvius
  • Mohi Ahmed
  • Chen Wang
  • Xuguang Nie
  • Christine E Seidman
  • Claire Niro
  • Julien Giordani
  • Ricardo D Coletta
  • Fabien Le Grand
  • Jinshu Xu
  • Claudia Compagnucci
  • Chaoshe Guo
  • Alexandre Blais
  • Evelyne Souil
  • Heide L Ford
  • Helmut Fuchs
  • Martin Hrabe de Angelis
  • J G Seidman
  • Michelle Self
  • Frederic F S Depreux
  • Christophe Houbron
  • Moises Mallo
  • Dan Liu
  • Zen ichi Ando
  • John N Griffin
  • Nissrine Daou
  • Alan W Leung
  • Fenglei He
  • Paul Delgado-Olguín
  • Michael J Depew
  • Aibin He
  • Chu An Wang
  • Shahragim Tajbakhsh
  • Claire Anderson
  • William T Pu
  • Julien Pujol
  • Anne Françoise Richard
  • Ye Sun
  • Jianbo Sun
  • Nicolas Sgarioto
  • Bernice E Morrow
  • Maryline Favier
  • Isabelle Guillet-Deniau
  • Tânia Vinagre
  • Nicholas A Fairbridge
  • Laina Freyer
  • Hiroshi Yajima
  • Guiyuan He
  • Philippe Daubas
  • Binglai Chen
  • Simon D Gerber
  • Morag A Lewis
  • Karen P Steel
  • Elizabeth Quint
  • Joy Kahn
  • Hong Zhang
  • Erika A Bosman
  • E L McCoy
  • Ryoichiro Kageyama
  • Torben Soker
  • Jolanta E Pitera
  • Guillermo Oliver
  • Ana Novoa
  • Nicoletta Bobola
  • Frederic Relaix
  • Hiroki Kobayashi
  • Deepali Pitre Poladia
  • Kevin T Bush
  • Yoshiyuki Konishi
  • Filipa Moraes
  • Eva Kutejova
  • Yoichiro Iwakura

Detail Information

Publications90

  1. doi Initiation of olfactory placode development and neurogenesis is blocked in mice lacking both Six1 and Six4
    Binglai Chen
    Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine of NYU, New York, NY 10029, USA
    Dev Biol 326:75-85. 2009
    ..In mice, loss of Six1 affects OE morphogenesis but not placode formation...
  2. ncbi Mutation in the transcriptional coactivator EYA4 causes dilated cardiomyopathy and sensorineural hearing loss
    Jost Schönberger
    Harvard Medical School, Department of Genetics, 77 Avenue Louis Pasteur, Boston, Massachusetts 02115, USA
    Nat Genet 37:418-22. 2005
    ..These data define unrecognized and crucial roles for Eya4-Six-mediated transcriptional regulation in normal heart function...
  3. ncbi Expression profiling identifies the cytoskeletal organizer ezrin and the developmental homeoprotein Six-1 as key metastatic regulators
    Yanlin Yu
    Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Nat Med 10:175-81. 2004
    ..VIL2 and SIX1 expression was enhanced in human RMS tissue, significantly correlating with clinical stage...
  4. pmc The Six1 homeoprotein stimulates tumorigenesis by reactivation of cyclin A1
    Ricardo D Coletta
    Department of Obstetrics and Gynecology, University Colorado Health Sciences Center, Denver, CO 80262, USA
    Proc Natl Acad Sci U S A 101:6478-83. 2004
    ..Here we show that the tissue-restricted cyclin A1 is a transcriptional target of the Six1 homeoprotein...
  5. pmc Expression of Six1 homeobox gene during development of the mouse submandibular salivary gland
    E L McCoy
    Program in Molecular Biology, University of Colorado School of Medicine, Denver, CO, USA
    Oral Dis 15:407-13. 2009
    ..Here we report the temporal and spatial expression of Six1 during maturation of the mouse submandibular salivary gland (SSG) from embryonic day 18.5 (E18...
  6. pmc Six1 and Eya1 are critical regulators of peri-cloacal mesenchymal progenitors during genitourinary tract development
    Chen Wang
    Department of Urology, Children s Hospital Boston, 300 Longwood Avenue, Harvard Medical School, Boston, MA 02115, USA
    Dev Biol 360:186-94. 2011
    The evolutionarily conserved Six1-Eya1 transcription complex is central to mammalian organogenesis, and deletion of these genes in mice results in developmental anomalies of multiple organs that recapitulate human branchio-oto-renal (BOR) ..
  7. pmc Eya4-deficient mice are a model for heritable otitis media
    Frederic F S Depreux
    Department of Genetics, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Clin Invest 118:651-8. 2008
    ..We suggest that some human otitis media susceptibility reflects underlying genetic predisposition in genes like EYA4 that regulate middle ear and eustachian tube anatomy...
  8. pmc Eya1 and Six1 are essential for early steps of sensory neurogenesis in mammalian cranial placodes
    Dan Zou
    McLaughlin Research Institute for Biomedical Sciences, 1520 23rd Street South, Great Falls, MT 59405, USA
    Development 131:5561-72. 2004
    ..It interacts with and functions upstream of the homeobox gene Six1 during otic placodal development. Here, we have examined their role in cranial sensory neurogenesis...
  9. pmc Six1 regulates stem cell repair potential and self-renewal during skeletal muscle regeneration
    Fabien Le Grand
    Institut National de la Santé et de la Recherche Médicale U1016, Institut Cochin, Paris 75014, France
    J Cell Biol 198:815-32. 2012
    ..we observe that adult quiescent SCs and their activated descendants expressed the homeodomain transcription factor Six1. Genetic disruption of Six1 specifically in adult SCs impaired myogenic cell differentiation, impaired myofiber ..
  10. pmc Discovery, optimization and validation of an optimal DNA-binding sequence for the Six1 homeodomain transcription factor
    Yubing Liu
    Ottawa Institute of Systems Biology and Biochemistry, Microbiology and Immunology Department, Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada
    Nucleic Acids Res 40:8227-39. 2012
    The Six1 transcription factor is a homeodomain protein involved in controlling gene expression during embryonic development. Six1 establishes gene expression profiles that enable skeletal myogenesis and nephrogenesis, among others...
  11. pmc Cooperation between myogenic regulatory factors and SIX family transcription factors is important for myoblast differentiation
    Yubing Liu
    Ottawa Institute of Systems Biology and Department of Biochemistry, Microbiology and Immunology, University of Ottawa, Faculty of Medicine, 451 Smyth Road, Ottawa, ON K1H 8M5, Canada
    Nucleic Acids Res 38:6857-71. 2010
    ..We have conducted a functional genomic study of the role played by SIX1 and SIX4 during the differentiation of skeletal myoblasts, a model of adult muscle regeneration...
  12. ncbi Six1 and Six4 homeoproteins are required for Pax3 and Mrf expression during myogenesis in the mouse embryo
    Raphaelle Grifone
    Departement Genetique, Developpement et Pathologie Moleculaire, Institut Cochin INSERM 567, CNRS UMR 8104, Universite Paris V, 24 Rue du Faubourg Saint Jacques 75014 Paris, France
    Development 132:2235-49. 2005
    In mammals, Six5, Six4 and Six1 genes are co-expressed during mouse myogenesis. Six4 and Six5 single knockout (KO) mice have no developmental defects, while Six1 KO mice die at birth and show multiple organ developmental defects...
  13. doi Regulation of Six1 expression by evolutionarily conserved enhancers in tetrapods
    Shigeru Sato
    Division of Biology, Center for Molecular Medicine, Jichi Medical University, Yakushiji, Shimotsuke, Tochigi 329 0498, Japan
    Dev Biol 368:95-108. 2012
    The Six1 homeobox gene plays critical roles in vertebrate organogenesis...
  14. pmc Six proteins regulate the activation of Myf5 expression in embryonic mouse limbs
    Julien Giordani
    Département de Génétique et Développement, Institut Cochin, Universite Paris Descartes, Paris, France
    Proc Natl Acad Sci U S A 104:11310-5. 2007
    ..5 kb from the Myf5 gene. In the present study we show that Myf5 expression is severely impaired in the limb buds of Six1(-/-) and Six1(-/-)Six4(-/+) mouse mutants despite the presence of myogenic progenitor cells...
  15. pmc EYA1 and SIX1 drive the neuronal developmental program in cooperation with the SWI/SNF chromatin-remodeling complex and SOX2 in the mammalian inner ear
    Mohi Ahmed
    Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine of NYU, New York, NY 10029, USA
    Development 139:1965-77. 2012
    ..Here, using loss- and gain-of-function models, we show that EYA1 and SIX1 are crucial otic neuronal determination factors upstream of NEUROG1 and NEUROD1...
  16. ncbi Six1 and Six4 are essential for Gdnf expression in the metanephric mesenchyme and ureteric bud formation, while Six1 deficiency alone causes mesonephric-tubule defects
    Hiroki Kobayashi
    Division of Integrative Cell Biology, Institute of Molecular Embryology and Genetics, Kumamoto University, 2 2 1 Honjo, Kumamoto 860 0811, Japan
    Mech Dev 124:290-303. 2007
    Interaction between the ureteric-bud epithelium and the metanephric mesenchyme is important for kidney development. Six1 and Six4 are the mammalian homologs of Drosophila sine oculis, and they are coexpressed in the nephrogenic mesenchyme...
  17. pmc Patterning of the third pharyngeal pouch into thymus/parathyroid by Six and Eya1
    Dan Zou
    McLaughlin Research Institute for Biomedical Sciences, Great Falls, MT 59405, USA
    Dev Biol 293:499-512. 2006
    ..Here, we examined their roles in pharyngeal pouch development. Six1-/- mice lack thymus and parathyroid and analysis of Six1-/- third pouch endoderm demonstrated that the patterning ..
  18. pmc Six2 is required for suppression of nephrogenesis and progenitor renewal in the developing kidney
    Michelle Self
    Department of Genetics and Tumor Cell Biology, St Jude Children s Research Hospital, Memphis, TN 38105 2794, USA
    EMBO J 25:5214-28. 2006
    ..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...
  19. pmc Eya1 regulates the growth of otic epithelium and interacts with Pax2 during the development of all sensory areas in the inner ear
    Dan Zou
    McLaughlin Research Institute for Biomedical Sciences, 1520 23rd Street South, MT 59405, USA
    Dev Biol 298:430-41. 2006
    ..However, it remains unknown whether Pax genes interact with Eya1 or Six1 during inner ear morphogenesis...
  20. ncbi Eya protein phosphatase activity regulates Six1-Dach-Eya transcriptional effects in mammalian organogenesis
    Xue Li
    Howard Hughes Medical Institute, School and Department of Medicine, UCSD, 9500 Gilman Drive, Room 345, La Jolla, California 92093 0648, USA
    Nature 426:247-54. 2003
    ..Here, we report that Six1 is required for the development of murine kidney, muscle and inner ear, and that it exhibits synergistic genetic ..
  21. doi Six family genes control the proliferation and differentiation of muscle satellite cells
    Hiroshi Yajima
    Division of Biology, Center for Molecular Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan
    Exp Cell Res 316:2932-44. 2010
    ..However, the mechanisms involved in their proliferation and differentiation remain elusive. Six1 and Six4 proteins were expressed in the nuclei of myofibers of adult mice and the numbers of myoblasts positive for ..
  22. pmc Expression of myogenin during embryogenesis is controlled by Six/sine oculis homeoproteins through a conserved MEF3 binding site
    F Spitz
    Institut National de la Santé et de la Recherche Médicale U129, Institut Cochin de Genetique Moleculaire, Universite Rene Descartes Paris V, 24 rue du Faubourg Saint Jacques, 75014 Paris, France
    Proc Natl Acad Sci U S A 95:14220-5. 1998
    ..Antibodies directed specifically against Six1 or Six4 proteins reveal that each of these proteins is present in the embryo when myogenin is activated and ..
  23. ncbi Eya1-deficient mice lack ears and kidneys and show abnormal apoptosis of organ primordia
    P X Xu
    Division of Genetics, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA
    Nat Genet 23:113-7. 1999
    ..In addition, our results suggest that an evolutionarily conserved Pax-Eya-Six regulatory hierarchy is used in mammalian ear and kidney development...
  24. pmc Six4, a putative myogenin gene regulator, is not essential for mouse embryonal development
    H Ozaki
    Departments of Biology, Jichi Medical School, Tochigi 329 0498, Japan
    Mol Cell Biol 21:3343-50. 2001
    ..The expression pattern was similar to that of Six1 except at the early stage of embryonic day 8.5...
  25. doi Six1 is indispensable for production of functional progenitor cells during olfactory epithelial development
    Keiko Ikeda
    Division of Biology, Center for Molecular Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan
    Int J Dev Biol 54:1453-64. 2010
    ..We previously reported that Six1(-/-) shows a lack of mature ORNs throughout development and disorganization of OE after E12.5...
  26. doi Pax6 regulates craniofacial form through its control of an essential cephalic ectodermal patterning center
    Claudia Compagnucci
    Department of Craniofacial Development, King s College London, Guy s Hospital, London SE1 9RT, United Kingdom
    Genesis 49:307-25. 2011
    ..lambdoidal junction, of secreted signaling factors (including Fgf8 and Bmp4) and transcription factors (including Six1 and Dlx5) essential for upper jaw and/or nasal capsular development...
  27. doi Six1 and Six4 gene expression is necessary to activate the fast-type muscle gene program in the mouse primary myotome
    Claire Niro
    Institut Cochin, Universite Paris Descartes, CNRS UMR 8104, Paris, France
    Dev Biol 338:168-82. 2010
    ..Affymetrix transcriptomal analysis of Six1(-/-)Six4(-/-) E10.5 somites revealed the specific down-regulation of many genes of the fast-type muscle program...
  28. pmc Molecular interaction and synergistic activation of a promoter by Six, Eya, and Dach proteins mediated through CREB binding protein
    Keiko Ikeda
    Department of Biology, Jichi Medical School, Yakushiji, Minamikawachi, Kawachi, Tochigi, 329 0498, Japan
    Mol Cell Biol 22:6759-66. 2002
    ..This work provides fundamental information on the role and the mechanism of action of this gene cassette in tissue differentiation and organogenesis...
  29. ncbi Altered myogenesis in Six1-deficient mice
    Christine Laclef
    Departement Genetique, Developpement et Pathologie Moleculaire, Institut Cochin INSERM 567, CNRS UMR 8104, Universite Paris V, 24 rue du Faubourg Saint Jacques, 75014 Paris, France
    Development 130:2239-52. 2003
    ..To determine the functions of the Six1 gene during myogenesis, we constructed Six1-deficient mice by replacing its first exon with the lacZ gene...
  30. pmc A Tbx1-Six1/Eya1-Fgf8 genetic pathway controls mammalian cardiovascular and craniofacial morphogenesis
    Chaoshe Guo
    Department of Urology, Children s Hospital Boston, and Department of Surgery and Pathology, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Clin Invest 121:1585-95. 2011
    ..Here, we have identified a genetic pathway involving the Six1/Eya1 transcription complex that regulates cardiovascular and craniofacial development...
  31. ncbi Thymus, kidney and craniofacial abnormalities in Six 1 deficient mice
    Christine Laclef
    Departement Genetique, Developpement et Pathologie Moleculaire, Universite Paris V, 24 rue du Faubourg Saint Jacques, 75014 Paris, France
    Mech Dev 120:669-79. 2003
    ..To determine the functions of the Six1 gene, we constructed Six1-deficient mice by replacing its first exon by the beta-galactosidase gene...
  32. doi Genesis of muscle fiber-type diversity during mouse embryogenesis relies on Six1 and Six4 gene expression
    Anne Françoise Richard
    Institut Cochin, Universite Paris Descartes, Sorbonne Paris Cité, CNRS UMR 8104, Paris, France
    Dev Biol 359:303-20. 2011
    ..Genesis of this fiber-type heterogeneity during development remains poorly known, at least in mammals. Six1 and Six4 homeoproteins of the Six/sine oculis family are expressed throughout muscle development in mice, and Six1 ..
  33. pmc The role of Six1 in mammalian auditory system development
    Weiming Zheng
    McLaughlin Research Institute for Biomedical Sciences, 1520 23rd Street South, Great Falls, MT 59405, USA
    Development 130:3989-4000. 2003
    ..However, their roles during auditory system development have not been studied. We report that Six1 is required for mouse auditory system development...
  34. ncbi Six1 is essential for early neurogenesis in the development of olfactory epithelium
    Keiko Ikeda
    Division of Biology, Center for Molecular Medicine, Jichi Medical University, Shimotsuke, Tochigi 329 0498, Japan
    Dev Biol 311:53-68. 2007
    ..Little is known about the molecular pathway of early neurogenesis. The homeodomain protein Six1 is expressed in all OP cells and neurogenic precursors in the OE...
  35. ncbi Six1 controls patterning of the mouse otic vesicle
    Hidenori Ozaki
    Division of Biology, Center for Molecular Medicine, Jichi Medical School, Tochigi 329 0498, Japan
    Development 131:551-62. 2004
    b>Six1 is a member of the Six family homeobox genes, which function as components of the Pax-Six-Eya-Dach gene network to control organ development...
  36. doi Expression of Six1 and Six4 in mouse taste buds
    Yuko Suzuki
    Division of Histology, Department of Oral Growth and Development, School of Dentistry, Health Sciences University of Hokkaido, Ishikari Tobetsu 061 0293, Japan
    J Mol Histol 41:205-14. 2010
    ..We examined the expression of Six1 and Six4 mRNAs in mouse taste buds by using in situ hybridization...
  37. ncbi Slc12a2 is a direct target of two closely related homeobox proteins, Six1 and Six4
    Zen ichi Ando
    Division of Biology, Center for Molecular Medicine, Jichi Medical School, Minamikawachi, Tochigi, Japan
    FEBS J 272:3026-41. 2005
    ..Of the six family members (Six1-Six6) in mice, Six1 and Six4 show similar expression patterns during embryogenesis...
  38. ncbi Six1 and Six4 promote survival of sensory neurons during early trigeminal gangliogenesis
    Yoshiyuki Konishi
    Division of Biology Center for Molecular Medicine, Jichi Medical University, 3311 1 Yakushiji, Shimotsuke, Tochigi 329 0498, Japan
    Brain Res 1116:93-102. 2006
    ..In the present study, we investigated the role of Six1 and Six4 in the development of trigeminal ganglia...
  39. pmc SIX1 mutations cause branchio-oto-renal syndrome by disruption of EYA1-SIX1-DNA complexes
    Rainer G Ruf
    Department of Pediatrics, University of Michigan, Ann Arbor, MI 48109, USA
    Proc Natl Acad Sci U S A 101:8090-5. 2004
    ..1. Within the 33-megabase critical genetic interval, we located the SIX1, SIX4, and SIX6 genes, which act within a genetic network of EYA and PAX genes to regulate organogenesis...
  40. ncbi Homeobox genes and connective tissue patterning
    G Oliver
    Department of Molecular Cell Biology, Max Planck Institute of Biophysical Chemistry, Gottingen, Germany
    Development 121:693-705. 1995
    ..Six 1 and Six 2 also are expressed in skeletal and smooth muscle, respectively. These genes may participate in the patterning of the distal tendons of the limb phalanges by setting positional values along the limb axes...
  41. pmc Six1 is required for the early organogenesis of mammalian kidney
    Pin Xian Xu
    McLaughlin Research Institute, 1520 23rd Street South, Great Falls, MT 59405, USA
    Development 130:3085-94. 2003
    ..to Drosophila sine oculis (so) which encodes a homeodomain transcription factor, is composed of six members (Six1-6)...
  42. pmc Six1 and Eya1 expression can reprogram adult muscle from the slow-twitch phenotype into the fast-twitch phenotype
    Raphaelle Grifone
    Departement Genetique, Developpement et Pathologie Moleculaire, Institut Cochin INSERM 567, CNRS UMR 8104, Universite Paris V, France
    Mol Cell Biol 24:6253-67. 2004
    ..We also show that among the Six and Eya gene products expressed in mouse skeletal muscle, Six1 and Eya1 proteins accumulate preferentially in the nuclei of fast-twitch muscles...
  43. ncbi Eya1 and Eya2 proteins are required for hypaxial somitic myogenesis in the mouse embryo
    Raphaelle Grifone
    département génétique et développement, Institut Cochin Paris, INSERM, U567, Paris, F 75014 France
    Dev Biol 302:602-16. 2007
    In mammals, Pax3, Six4, Six1 and Six5 genes are co-expressed with Eya1, Eya2 and Eya4 genes during mouse somitogenesis...
  44. doi Six1 and Six1 cofactor expression is altered during early skeletal muscle overload in mice
    Bradley S Gordon
    Department of Exercise Science, Public Health Research Center, University of South Carolina, 3rd Floor, 921 Assembly Street, Columbia, SC 29208, USA
    J Physiol Sci 62:393-401. 2012
    b>Six1 is a transcription factor that, along with cofactors (Eya1, Eya3, and Dach2), regulates skeletal muscle fiber-type and development...
  45. doi Cecr2 mutations causing exencephaly trigger misregulation of mesenchymal/ectodermal transcription factors
    Nicholas A Fairbridge
    Department of Biological Sciences, University of Alberta, Edmonton, Canada
    Birth Defects Res A Clin Mol Teratol 88:619-25. 2010
    ..Over 200 mouse genes are associated with neural tube defects (NTDs), including Cecr2, the bromodomain-containing subunit of the CERF chromatin remodeling complex...
  46. doi Sine oculis homeobox homolog 1 promotes DNA replication and cell proliferation in cervical cancer
    Dan Liu
    Cancer Biology Research Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, P R China
    Int J Oncol 45:1232-40. 2014
    ..Here we report that Sine oculis homeobox homolog 1 (SIX1) functions as a master regulator in DNA replication of cervical cancer cells...
  47. doi A new role for the calcineurin/NFAT pathway in neonatal myosin heavy chain expression via the NFATc2/MyoD complex during mouse myogenesis
    Nissrine Daou
    Centre d Etude de la Sensori Motricité, UMR 8194 CNRS, Universite Paris Descartes, Centre Universitaire des Saints Peres, 45 Rue des Saints Peres, F 75270 Paris Cedex 06, France
    Development 140:4914-25. 2013
    ..Altogether, our findings demonstrate that the calcineurin/NFAT pathway plays a new role in establishing the early muscle fiber type in immature myofibers during embryogenesis. ..
  48. ncbi Identification of an evolutionarily conserved, functional noncoding element regulated by Six1 homeoprotein
    Yongsu Jeong
    Department of Genetic Engineering, College of Life Sciences and Graduate School of Biotechnology, Kyung Hee University, Yongin si, Republic of Korea
    Genes Genet Syst 85:233-40. 2010
    b>Six1, which belongs to the sine oculis homeobox (Six) protein family, is an evolutionarily conserved transcription factor found in diverse organisms ranging from flatworms to humans...
  49. pmc A critical role for PDGFRα signaling in medial nasal process development
    Fenglei He
    Department of Developmental and Regenerative Biology, Icahn School of Medicine at Mount Sinai, New York, New York, United States of America
    PLoS Genet 9:e1003851. 2013
    ..We thus establish PDGFRα as a novel regulator of MNP development and elucidate the roles of its downstream signaling pathways at cellular and molecular levels. ..
  50. pmc Differential BMP signaling controls formation and differentiation of multipotent preplacodal ectoderm progenitors from human embryonic stem cells
    Alan W Leung
    Department of Genetics and Developmental Biology, University of Connecticut Health Center, 400 Farmington Avenue, Farmington, CT 06030 6403, USA
    Dev Biol 379:208-20. 2013
    ..anterior neural plate, and expresses a unique set of evolutionarily conserved transcription regulators including Six1, Eya 1 and Eya2...
  51. pmc Eya1-Six1 interaction is sufficient to induce hair cell fate in the cochlea by activating Atoh1 expression in cooperation with Sox2
    Mohi Ahmed
    Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine, New York, NY 10029, USA
    Dev Cell 22:377-90. 2012
    Inner-ear hair cell differentiation requires Atoh1 function, while Eya1, Six1, and Sox2 are coexpressed in sensory progenitors and mutations in these genes cause sensorineural hearing loss...
  52. pmc SIX1 induces lymphangiogenesis and metastasis via upregulation of VEGF-C in mouse models of breast cancer
    Chu An Wang
    Program in Molecular Biology, University of Colorado Denver School of Medicine, Aurora, Colorado 80045, USA
    J Clin Invest 122:1895-906. 2012
    ..We therefore investigated whether SIX1, a homeodomain-containing transcription factor previously associated in breast cancer with lymph node positivity, ..
  53. pmc Embryonic origin and remodeling of the urinary and digestive outlets
    Chen Wang
    Department of Urology, Boston Children s Hospital, Boston, Massachusetts, USA
    PLoS ONE 8:e55587. 2013
    ..We demonstrate that Six1 and Six2 are complementarily but asymmetrically expressed in the PCM progenitors...
  54. pmc Fgf8 dosage determines midfacial integration and polarity within the nasal and optic capsules
    John N Griffin
    Dept of Craniofacial Development, King s College London, Floor 27, Guy s Hospital, London Bridge, London SE1 9RT, UK
    Dev Biol 374:185-97. 2013
    ..Taken together, our data highlight Fgf8 signaling in craniofacial development as a plausible target for evolutionary selective pressures...
  55. pmc Sonic hedgehog acts cell-autonomously on muscle precursor cells to generate limb muscle diversity
    Claire Anderson
    Department of Biomedical Science, University of Sheffield, Sheffield, United Kingdom
    Genes Dev 26:2103-17. 2012
    ..Thus, Shh production in the limb ZPA is essential for the spatiotemporal control of myogenesis and coordinates muscle and skeletal development by acting directly to regulate the formation of specific ventral muscles...
  56. pmc Development of gustatory papillae in the absence of Six1 and Six4
    Yuko Suzuki
    Division of Biostatistics, Department of Clinical Psychology, School of Psychological Science, Health Sciences University of Hokkaido, Ishikari Tobetsu, Japan
    J Anat 219:710-21. 2011
    ..In a previous paper, Six1 was reported to be expressed in the taste bud-bearing lingual papillae of mice, and loss of Six1 affected the ..
  57. ncbi Role of fibroblast growth factor receptors 1 and 2 in the metanephric mesenchyme
    Deepali Pitre Poladia
    Center for Cell and Vascular Biology, Columbus Children s Research Institute, 700 Children s Drive, Columbus, OH 43205, USA
    Dev Biol 291:325-39. 2006
    ..By in situ hybridization, regions of mutant mesenchyme near the ureteric bud(s) express Eya1 and Six1, but not Six2, Sall1, or Pax2, while the ureteric bud expresses Ret and Pax2 normally...
  58. pmc Polycomb repressive complex 2 regulates normal development of the mouse heart
    Aibin He
    Department of Cardiology, Children s Hospital Boston, MA, USA
    Circ Res 110:406-15. 2012
    ..Polycomb repressive complex 2 (PRC2) trimethylates histone H3 at lysine 27, which establishes H3K27me3 repressive epigenetic marks that promote tissue-specific differentiation by silencing ectopic gene programs...
  59. pmc Epigenetic repression of cardiac progenitor gene expression by Ezh2 is required for postnatal cardiac homeostasis
    Paul Delgado-Olguín
    Gladstone Institute of Cardiovascular Disease, San Francisco, California, USA
    Nat Genet 44:343-7. 2012
    ..cardiac gene expression and prevents cardiac pathology by repressing the homeodomain transcription factor gene Six1, which functions in cardiac progenitor cells but is stably silenced upon cardiac differentiation...
  60. pmc A comparative study of Eya1 and Eya4 protein function and its implication in branchio-oto-renal syndrome and DFNA10
    Yuzhou Zhang
    Molecular Otolaryngology Research Laboratories, University of Iowa, Iowa City, IA 52242, USA
    J Assoc Res Otolaryngol 5:295-304. 2004
    ..We verified that bait constructs of the homologous region ( Eya1HR and Eya4HR) interact with Six1 prey constructs, although no interaction with Dach1 prey was demonstrable...
  61. pmc Betaglycan is required for the establishment of nephron endowment in the mouse
    Kenneth A Walker
    Department of Anatomy and Developmental Biology, Monash University, Clayton, Victoria, Australia
    PLoS ONE 6:e18723. 2011
    ..5 and e14.5. The opposing morphological and molecular phenotypes in betaglycan heterozygote and null mutants demonstrate that the levels of betaglycan must be tightly regulated for optimal kidney development...
  62. pmc Six1 regulates Grem1 expression in the metanephric mesenchyme to initiate branching morphogenesis
    Xuguang Nie
    Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine of NYU, New York, NY 10029, USA
    Dev Biol 352:141-51. 2011
    ..However, the mechanisms that regulate the GREM1-BMP4 signaling are unknown. Previous studies have shown that Six1-deficient mice lack kidneys, but form ureters...
  63. ncbi Cloning of the human SIX1 gene and its assignment to chromosome 14
    C A Boucher
    Genetics Unit, Department of Anatomy, Charing Cross and Westminster Medical School, Hammersmith, London, W6 8RF, UK
    Genomics 33:140-2. 1996
    The recently described murine homeobox genes, Six1 and Six2, which are expressed during development in limb tendons, have also been shown to be expressed in skeletal and smooth muscle, respectively...
  64. ncbi Development and differentiation of the ureteric bud into the ureter in the absence of a kidney collecting system
    Kevin T Bush
    Department of Medicine, University of California, San Diego, 9500 Gilman Drive, La Jolla, CA 92093 0693, and Urological Diseases Research Center, Department of Urology, Children s Hospital, Boston, MA 02115, USA
    Dev Biol 298:571-84. 2006
    b>Six1-/- mice were found to have apparently normal ureters in the absence of a kidney, suggesting that the growth and development of the unbranched ureter is largely independent of the more proximal portions of the UB which differentiates ..
  65. ncbi Identification and expression of six family genes in mouse retina
    K Kawakami
    Department of Biology, Jichi Medical School, Tochigi ken, Japan
    FEBS Lett 393:259-63. 1996
    ..Six5 and Six2 bind to the same sequence as does AREC3/Six4, while Six3 does not. These observations suggest that some of the Six family genes can regulate the same target genes...
  66. ncbi Tissue and developmental distribution of Six family gene products
    H Ohto
    Department of Biology, Jichi Medical School, Tochigi, Japan
    Int J Dev Biol 42:141-8. 1998
    ..Our results suggest that Six4 plays a specific role in the differentiation or maturation of neuronal cells, while Six5 is an adult type Six gene isoform product and is distributed in the kidney, liver and lung...
  67. ncbi Molecular effects of Eya1 domain mutations causing organ defects in BOR syndrome
    C Buller
    McLaughlin Research Institute for Biomedical Sciences, 1520 23rd Street South, Great Falls, MT 59405, USA
    Hum Mol Genet 10:2775-81. 2001
    ..However, four mutations are crucial for protein-protein interactions in both yeast and mammalian cells. Our results provide insights into the molecular mechanisms of organ defects detected in human syndromes...
  68. ncbi Identification of transcriptional targets for Six5: implication for the pathogenesis of myotonic dystrophy type 1
    Shigeru Sato
    Department of Biology, Jichi Medical School, Minamikawachi, Tochigi 329 0498, Japan
    Hum Mol Genet 11:1045-58. 2002
    ..Our results not only identify Six5 as an activator that directs Igfbp5 expression but also suggest that reduced SIX5 expression in DM1 might contribute to specific aspects of the DM1 phenotype...
  69. pmc Eya1 is required for the morphogenesis of mammalian thymus, parathyroid and thyroid
    Pin Xian Xu
    McLaughlin Research Institute for Biomedical Sciences, Great Falls, MT 59405, USA
    Development 129:3033-44. 2002
    ..These data indicate that Eya1 also regulates mature thyroid gland formation. Furthermore, we show that Six1 expression is markedly reduced in the arch mesenchyme, pouch endoderm and surface ectoderm in the pharyngeal region ..
  70. ncbi Establishment of tendon-derived cell lines exhibiting pluripotent mesenchymal stem cell-like property
    R Salingcarnboriboon
    Department of Molecular Pharmacology, Medical Research Institute, Tokyo Medical and Dental University, Tokyo, Japan
    Exp Cell Res 287:289-300. 2003
    ..Tendon phenotype-related genes such as those encoding scleraxis, Six1, EphA4, COMP, and type I collagen were expressed in these tendon cell clones...
  71. ncbi Partially overlapping expression of Gata2 and Gata3 during inner ear development
    Kersti Lilleväli
    Institute of Biotechnology, University of Helsinki, Helsinki, Finland
    Dev Dyn 231:775-81. 2004
    ..5 and Gata3 expression was unchanged. In contrast, a delay and strong reduction of Gata2 expression was detected in Gata3-/- otic epithelium...
  72. ncbi Tbx1 is required for proper neural crest migration and to stabilize spatial patterns during middle and inner ear development
    Filipa Moraes
    Instituto Gulbenkian de Ciencia, UT, Rua da Quinta Grande 6, 2780 156 Oeiras, Portugal
    Mech Dev 122:199-212. 2005
    ..The inability of the Tbx1(-/-) embryos to keep properly segregated functional domains in the otocyst is likely the cause of the strong inner ear phenotypes observed in these mutants...
  73. ncbi Six1 is not involved in limb tendon development, but is expressed in limb connective tissue under Shh regulation
    Marie Ange Bonnin
    Biologie du Développement, CNRS UMR 7622, Universite P et M Curie, 9 quai Saint Bernard, Bat C, 6 e E, Case 24, Paris Cedex 05 75252, France
    Mech Dev 122:573-85. 2005
    Mice deficient for the homeobox gene Six1 display defects in limb muscles consistent with the Six1 expression in myogenic cells...
  74. ncbi Transcriptional activation of the SALL1 by the human SIX1 homeodomain during kidney development
    Li Chai
    Department of Pathology, Brigham and Women s Hospital Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 281:18918-26. 2006
    ..Consensus binding sites were identified for several transcription factors, with multiple sites for WT1 and SIX1. In transient transfection assays, SALL1 promoter activity was higher in HEK-293 human kidney cells and COS-7 ..
  75. doi Inactivation of Six2 in mouse identifies a novel genetic mechanism controlling development and growth of the cranial base
    Guiyuan He
    School of Dentistry, Faculty of Medical and Human Sciences, University of Manchester, Manchester M13 9PT, UK
    Dev Biol 344:720-30. 2010
    ..The comparable expression during human embryogenesis and the high protein conservation from mouse to human implicate SIX2 loss-of-function as a potential congenital cause of anterior cranial base defects in humans...
  76. pmc Ski regulates muscle terminal differentiation by transcriptional activation of Myog in a complex with Six1 and Eya3
    Hong Zhang
    Department of Biochemistry, Case Western Reserve University, Cleveland, OH 44106, USA
    J Biol Chem 284:2867-79. 2009
    ..Transactivation of Myog was largely dependent on a MEF3 site bound by Six1, not on the binding site of MyoD or MEF2...
  77. pmc Pleiotropic effects in Eya3 knockout mice
    Torben Soker
    Helmholtz Center Munich, German Research Center for Environmental Health, Institute of Developmental Genetics, Neuherberg, Germany
    BMC Dev Biol 8:118. 2008
    ..The functions of its mammalian orthologs Eya1-4 are only partially understood and no mouse model exists for Eya3. Therefore, we characterized the phenotype of a new Eya3 knockout mouse mutant...
  78. pmc An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice
    Morag A Lewis
    Wellcome Trust Sanger Institute, Hinxton, UK
    Nat Genet 41:614-8. 2009
    ..This is the first microRNA found associated with deafness, and diminuendo represents a model for understanding and potentially moderating progressive hair cell degeneration in hearing loss more generally...
  79. pmc Catweasel mice: a novel role for Six1 in sensory patch development and a model for branchio-oto-renal syndrome
    Erika A Bosman
    The Wellcome Trust Sanger Institute, The Wellcome Trust Genome Campus, Hinxton CB10 1SA, UK
    Dev Biol 328:285-96. 2009
    ..We mapped the catweasel mutation to a critical region of 13 Mb on chromosome 12 containing the Six1, -4 and -6 genes...
  80. doi Muscle contraction is necessary to maintain joint progenitor cell fate
    Joy Kahn
    Department of Molecular Genetics, Weizmann Institute of Science, Rehovot, Israel
    Dev Cell 16:734-43. 2009
    ..In conclusion, our findings provide the missing link between progenitor cell fate determination and embryonic movement, two processes shown to be essential for correct organogenesis...
  81. doi The murine Fgfrl1 receptor is essential for the development of the metanephric kidney
    Simon D Gerber
    Department of Clinical Research, University of Bern, 3010 Bern, Switzerland
    Dev Biol 335:106-19. 2009
    ..We also observed a loss of Pax2 positive nephron precursor cells and an increase of apoptosis in the cortical zone of the remnant kidney. Fgfrl1 is therefore essential for mesenchymal differentiation in the early steps of nephrogenesis...
  82. pmc Characterization of the Six1 homeobox gene in normal mammary gland morphogenesis
    Ricardo D Coletta
    Department of Obstetrics and Gynecology, University of Colorado Denver, Anschutz Medical Campus, 12800 E, 19th Ave, Aurora, CO 80045, USA
    BMC Dev Biol 10:4. 2010
    The Six1 homeobox gene is highly expressed in the embryonic mammary gland, continues to be expressed in early postnatal mammary development, but is lost when the mammary gland differentiates during pregnancy...
  83. doi Evidence for a myotomal Hox/Myf cascade governing nonautonomous control of rib specification within global vertebral domains
    Tânia Vinagre
    Instituto Gulbenkian de Ciencia, Rua da Quinta Grande 6, 2780 156 Oeiras, Portugal
    Dev Cell 18:655-61. 2010
    ..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...
  84. doi Conserved expression of mouse Six1 in the pre-placodal region (PPR) and identification of an enhancer for the rostral PPR
    Shigeru Sato
    Division of Biology, Center for Molecular Medicine, Jichi Medical University, 3311 1 Yakushiji, Shimotsuke, Tochigi 329 0498, Japan
    Dev Biol 344:158-71. 2010
    ..all placodes originate from a common precursor domain, the pre-placodal region (PPR), marked by the expression of Six1/4 and Eya1/2...
  85. pmc Canonical Wnt signaling modulates Tbx1, Eya1, and Six1 expression, restricting neurogenesis in the otic vesicle
    Laina Freyer
    Department of Genetics, Albert Einstein College of Medicine, Bronx, New York, USA
    Dev Dyn 239:1708-22. 2010
    ..canonical Wnt signaling sets boundaries for pattern formation in the otic vesicle (OV), we examined Tbx1 and Eya1-Six1 downstream of activated beta-catenin...
  86. pmc Fras1, a basement membrane-associated protein mutated in Fraser syndrome, mediates both the initiation of the mammalian kidney and the integrity of renal glomeruli
    Jolanta E Pitera
    Nephro Urology Unit, UCL Institute of Child Health, London WC1 N 1EH, UK
    Hum Mol Genet 17:3953-64. 2008
    ..Fras1 deficiency causes defective interactions between the bud and mesenchyme, correlating with disturbed expression of key nephrogenic molecules. Furthermore, Fras1 may also be required for the formation of normal glomeruli...
  87. pmc Eya 1 acts as a critical regulator for specifying the metanephric mesenchyme
    Gangadharan Sajithlal
    McLaughlin Research Institute for Biomedical Sciences, Great Falls, MT 59405, USA
    Dev Biol 284:323-36. 2005
    ....
  88. ncbi Hoxa2 downregulates Six2 in the neural crest-derived mesenchyme
    Eva Kutejova
    Department of Developmental Biology, Max Planck Institute of Immunobiology, Stuebeweg 51, 79108 Freiburg, Germany
    Development 132:469-78. 2005
    ..Furthermore, we demonstrate that Hoxa2 regulation of Six2 is confined to a 0.9 kb fragment of the Six2 promoter and that Hoxa2 binds to this promoter region. These results strongly suggest that Six2 is a direct target of Hoxa2...
  89. ncbi Lmx1b expression during joint and tendon formation: localization and evaluation of potential downstream targets
    Sandra D Dreyer
    Department of Pathology and Human Anatomy, Loma Linda University, 24785 Stewart St, Loma Linda, CA 92350, USA
    Gene Expr Patterns 4:397-405. 2004
    ..to developing joints and associated tissues in normal and Lmx1b knockout (KO) mice including: Gdf-5, sFrp2, sFrp3, Six1 and Six2. Lmx1b was diffusely expressed in the undifferentiated dorsal mesoderm of the emerging limb bud (E9.5-E11...
  90. pmc SIX1 acts synergistically with TBX18 in mediating ureteral smooth muscle formation
    Xuguang Nie
    Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine of New York University, New York, NY 10029, USA
    Development 137:755-65. 2010
    ..b>Six1 is a crucial regulator of renal development: mutations in human SIX1 cause branchio-oto-renal (BOR) syndrome and ..

Research Grants1

  1. Hsal 2, A Novel Homeobox Gene in Hematopoiesis
    Li Chai; Fiscal Year: 2007
    ..In addition, a further objective of this proposal is to serve as a vehicle for the development of the candidate into an independent and productive investigator in the area of hematopoiesis and leukemogenesis. ..