Wt1

Summary

Gene Symbol: Wt1
Description: Wilms tumor 1 homolog
Alias: D630046I19Rik, Wt-1, Wilms tumor protein homolog
Species: mouse

Top Publications

  1. pmc GFRalpha1 is an essential receptor component for GDNF in the developing nervous system and kidney
    G Cacalano
    Department of Molecular Biology, Genentech, Incorporated, South San Francisco, California 94080, USA
    Neuron 21:53-62. 1998
  2. pmc Deregulated expression of the homeobox gene Cux-1 in transgenic mice results in downregulation of p27(kip1) expression during nephrogenesis, glomerular abnormalities, and multiorgan hyperplasia
    Aric W Ledford
    Department of Biology, East Carolina University, Greenville, NC 27858, USA
    Dev Biol 245:157-71. 2002
  3. ncbi Insights into the physiological role of WT1 from studies of genetically modified mice
    Maria Teresa Discenza
    Department of Biochemistry, McGill University, Montreal, Quebec H3G 1Y6, Canada
    Physiol Genomics 16:287-300. 2004
  4. ncbi WT-1 is required for early kidney development
    J A Kreidberg
    Whitehead Institute for Biomedical Research Cambridge, Massachusetts 02142
    Cell 74:679-91. 1993
  5. ncbi Wt1 and retinoic acid signaling are essential for stellate cell development and liver morphogenesis
    A IJpenberg
    MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK
    Dev Biol 312:157-70. 2007
  6. ncbi Defective glomerulogenesis in the absence of laminin alpha5 demonstrates a developmental role for the kidney glomerular basement membrane
    J H Miner
    Renal Division, Department of Internal Medicine, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, Missouri, 63110, USA
    Dev Biol 217:278-89. 2000
  7. ncbi FGF8 is required for cell survival at distinct stages of nephrogenesis and for regulation of gene expression in nascent nephrons
    Uta Grieshammer
    Department of Anatomy and Program in Developmental Biology, School of Medicine, University of California at San Francisco, San Francisco, CA 94143 2711, USA
    Development 132:3847-57. 2005
  8. pmc Wt1 controls retinoic acid signalling in embryonic epicardium through transcriptional activation of Raldh2
    Juan Antonio Guadix
    Department of Animal Biology, University of Malaga, E29071 Malaga, Spain
    Development 138:1093-7. 2011
  9. pmc Wilms tumor 1 (WT1) regulates KRAS-driven oncogenesis and senescence in mouse and human models
    Silvestre Vicent
    Cancer Biology Program, Department of Pediatrics, Stanford University School of Medicine, Stanford, California, USA
    J Clin Invest 120:3940-52. 2010
  10. ncbi The serosal mesothelium is a major source of smooth muscle cells of the gut vasculature
    Bettina Wilm
    Stahlman Cardiovascular Laboratories, Department of Medicine and Program for Developmental Biology, Vanderbilt University Medical Center, Nashville, TN 37232, USA
    Development 132:5317-28. 2005

Scientific Experts

Detail Information

Publications143 found, 100 shown here

  1. pmc GFRalpha1 is an essential receptor component for GDNF in the developing nervous system and kidney
    G Cacalano
    Department of Molecular Biology, Genentech, Incorporated, South San Francisco, California 94080, USA
    Neuron 21:53-62. 1998
    ....
  2. pmc Deregulated expression of the homeobox gene Cux-1 in transgenic mice results in downregulation of p27(kip1) expression during nephrogenesis, glomerular abnormalities, and multiorgan hyperplasia
    Aric W Ledford
    Department of Biology, East Carolina University, Greenville, NC 27858, USA
    Dev Biol 245:157-71. 2002
    ..Transient transfection experiments revealed that Cux-1 is an inhibitor of p27 promoter activity. These results suggest that Cux-1 regulates cell proliferation during early nephrogenesis by inhibiting expression of p27...
  3. ncbi Insights into the physiological role of WT1 from studies of genetically modified mice
    Maria Teresa Discenza
    Department of Biochemistry, McGill University, Montreal, Quebec H3G 1Y6, Canada
    Physiol Genomics 16:287-300. 2004
    The identification of WT1 gene mutations in children with WAGR and Denys-Drash syndromes pointed toward a role for WT1 in genitourinary system development...
  4. ncbi WT-1 is required for early kidney development
    J A Kreidberg
    Whitehead Institute for Biomedical Research Cambridge, Massachusetts 02142
    Cell 74:679-91. 1993
    ..In addition, the mutation caused abnormal development of the mesothelium, heart, and lungs. Our results establish a crucial role for WT-1 in early urogenital development...
  5. ncbi Wt1 and retinoic acid signaling are essential for stellate cell development and liver morphogenesis
    A IJpenberg
    MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK
    Dev Biol 312:157-70. 2007
    Previous studies of knock-out mouse embryos have shown that the Wilms' tumor suppressor gene (Wt1) is indispensable for the development of kidneys, gonads, heart, adrenals and spleen...
  6. ncbi Defective glomerulogenesis in the absence of laminin alpha5 demonstrates a developmental role for the kidney glomerular basement membrane
    J H Miner
    Renal Division, Department of Internal Medicine, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, Missouri, 63110, USA
    Dev Biol 217:278-89. 2000
    ....
  7. ncbi FGF8 is required for cell survival at distinct stages of nephrogenesis and for regulation of gene expression in nascent nephrons
    Uta Grieshammer
    Department of Anatomy and Program in Developmental Biology, School of Medicine, University of California at San Francisco, San Francisco, CA 94143 2711, USA
    Development 132:3847-57. 2005
    ..Thus, unlike other FGF family members, which regulate growth and branching morphogenesis of the collecting duct system, Fgf8 encodes a factor essential for gene regulation and cell survival at distinct steps in nephrogenesis...
  8. pmc Wt1 controls retinoic acid signalling in embryonic epicardium through transcriptional activation of Raldh2
    Juan Antonio Guadix
    Department of Animal Biology, University of Malaga, E29071 Malaga, Spain
    Development 138:1093-7. 2011
    ..In the current study, we show that Wt1-null epicardial cells display decreased expression of Raldh2 both in vivo and in vitro...
  9. pmc Wilms tumor 1 (WT1) regulates KRAS-driven oncogenesis and senescence in mouse and human models
    Silvestre Vicent
    Cancer Biology Program, Department of Pediatrics, Stanford University School of Medicine, Stanford, California, USA
    J Clin Invest 120:3940-52. 2010
    ..of functional genomics and mouse genetics, we identified a role for the transcription factor Wilms tumor 1 (WT1) as a critical regulator of senescence and proliferation downstream of oncogenic KRAS signaling...
  10. ncbi The serosal mesothelium is a major source of smooth muscle cells of the gut vasculature
    Bettina Wilm
    Stahlman Cardiovascular Laboratories, Department of Medicine and Program for Developmental Biology, Vanderbilt University Medical Center, Nashville, TN 37232, USA
    Development 132:5317-28. 2005
    ..gut is initially devoid of a mesothelium but that serosal mesothelial cells expressing the Wilm's tumor protein (Wt1) move to and over the gut. Subsequently, a subset of these cells undergoes EMT and migrates throughout the gut...
  11. ncbi Murine homolog of SALL1 is essential for ureteric bud invasion in kidney development
    R Nishinakamura
    Division of Stem Cell Regulation, Institute of Medical Science, The University of Tokyo, Japan
    Development 128:3105-15. 2001
    ..Sall1 is therefore essential for ureteric bud invasion, the initial key step for metanephros development...
  12. ncbi YAC transgenic analysis reveals Wilms' tumour 1 gene activity in the proliferating coelomic epithelium, developing diaphragm and limb
    A W Moore
    MRC Human Genetics Unit, Western General Hospital, Edinburg, UK
    Mech Dev 79:169-84. 1998
    Wilms' Tumour 1 gene (WT1) is required for the correct development of the urogenital system...
  13. 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...
  14. 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...
  15. ncbi Pax2 and pax8 regulate branching morphogenesis and nephron differentiation in the developing kidney
    Melina Narlis
    McGill Cancer Centre and Biochemistry Department, McGill University, 3655 Promenade Sir William Osler, Montreal, Quebec H3G 1Y6, Canada
    J Am Soc Nephrol 18:1121-9. 2007
    ..Together, these results demonstrate a crucial role for Pax2 and Pax8 in nephron differentiation and branching morphogenesis of the metanephros...
  16. doi Abnormal epithelial cell polarity and ectopic epidermal growth factor receptor (EGFR) expression induced in Emx2 KO embryonic gonads
    Masatomo Kusaka
    Division for Sex Differentiation, Center for Transgenic Animals and Plants, National Institute for Basic Biology, National Institutes of Natural Sciences, Okazaki 444 8787, Japan
    Endocrinology 151:5893-904. 2010
    ..Our results strongly suggest that Emx2 is required for regulation of tight junction assembly and allowing migration of the gonadal epithelia to the mesenchyme, which are possibly mediated by suppression of Egfr expression...
  17. ncbi Epithelial transformation of metanephric mesenchyme in the developing kidney regulated by Wnt-4
    K Stark
    Department of Molecular and Cellular Biology, Harvard University, Cambridge, Massachusetts 02138
    Nature 372:679-83. 1994
    ..Thus, Wnt-4 appears to act as an autoinducer of the mesenchyme to epithelial transition that underlies nephron development...
  18. ncbi A far upstream cis-element is required for Wilms' tumor-1 (WT1) gene expression in renal cell culture
    H Scholz
    Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02215, USA
    J Biol Chem 272:32836-46. 1997
    ..novel cis-regulatory elements responsible for the tissue-restricted expression pattern of the Wilms' tumor-1 (WT1) gene, we mapped a total of 11 DNase I-hypersensitive sites in the 5'-flanking region and first intron of the human ..
  19. ncbi Pod-1/Capsulin shows a sex- and stage-dependent expression pattern in the mouse gonad development and represses expression of Ad4BP/SF-1
    M Tamura
    Mammalian Development Laboratory, Genetic Strains Research Center, National Institute of Genetics, Mishima, Japan
    Mech Dev 102:135-44. 2001
    ..These results suggest that Pod-1/Capsulin may play important roles in the development and sex differentiation of the mammalian gonad via transcriptional regulation of Ad4BP/SF-1...
  20. ncbi Genetic interactions between the Wilms' tumor 1 gene and the p53 gene
    Aswin L Menke
    Central Hematologie Laboratory, University Medical Centre St Radboud, 6500 HB, Nijmegen, The Netherlands
    Cancer Res 62:6615-20. 2002
    In recent years, a number of proteins have been identified that can modify the activities of the Wilms' Tumor 1 (WT1) proteins. One of these modifiers is the p53 protein...
  21. ncbi The LIM homeobox gene Lhx9 is essential for mouse gonad formation
    O S Birk
    Laboratory of Mammalian Genes and Development, National Institute of Child Health and Human Development, NIH, Bethesda, Maryland 20892, USA
    Nature 403:909-13. 2000
    ..Unlike mice lacking other genes that mediate early stages of gonadogenesis, Lhx9 mutants do not exhibit additional major developmental defects. Thus, LHX9 mutations may underlie certain forms of isolated gonadal agenesis in humans...
  22. ncbi The Wilms' tumour protein (WT1) shuttles between nucleus and cytoplasm and is present in functional polysomes
    Martina Niksic
    MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK
    Hum Mol Genet 13:463-71. 2004
    Mutations of the Wilms' tumour-1 (WT1) gene in humans can lead to childhood kidney cancer, life-threatening glomerular nephropathy and gonadal dysgenesis...
  23. doi Use of dual section mRNA in situ hybridisation/immunohistochemistry to clarify gene expression patterns during the early stages of nephron development in the embryo and in the mature nephron of the adult mouse kidney
    Kylie Georgas
    Institute for Molecular Bioscience, University of Queensland, St Lucia, QLD, 4072, Australia
    Histochem Cell Biol 130:927-42. 2008
    ..We have analysed in detail the protein expression of Wt1, Calb1 Aqp1, Aqp2 and Umod using these antibodies...
  24. ncbi Pax 2/8-regulated Gata 3 expression is necessary for morphogenesis and guidance of the nephric duct in the developing kidney
    David Grote
    McGill Cancer Centre and Biochemistry Department, McGill University, 3655 Promenade Sir William Osler, Montreal, Quebec H3G 1Y6, Canada
    Development 133:53-61. 2006
    ..Together, these results define Gata3 as a key regulator of nephric duct morphogenesis and guidance in the pro/mesonephric kidney...
  25. ncbi Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome
    J Pelletier
    Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, 02139
    Cell 67:437-47. 1991
    ..To investigate its possible role, we have analyzed the coding exons of the Wilms' tumor suppressor gene (WT1) for germline mutations...
  26. ncbi In situ expression of the early growth response gene-1 during murine nephrogenesis
    R R Rackley
    Department of Urology, Cleveland Clinic Foundation, Ohio, USA
    J Urol 154:700-5. 1995
    b>WT1 maps to chromosome 11p13 and encodes a deoxyribonucleic acid (DNA) binding protein whose expression is necessary for normal urogenital development...
  27. ncbi Initial differentiation of the metanephric mesenchyme is independent of WT1 and the ureteric bud
    M J Donovan
    Department of Pathology, Children s Hospital, Boston, MA 02115, USA
    Dev Genet 24:252-62. 1999
    ..The Wilms' tumor suppressor gene, Wt1, is required for mesenchymal cells to complete this developmental program...
  28. ncbi Two splice variants of the Wilms' tumor 1 gene have distinct functions during sex determination and nephron formation
    A Hammes
    MDC for Molecular Medicine, Developmental Genetics, Robert Rossle Str 10, 13092 Berlin, Germany
    Cell 106:319-29. 2001
    Alternative splicing of Wt1 results in the insertion or omission of the three amino acids KTS between zinc fingers 3 and 4. In vitro experiments suggest distinct molecular functions for + and -KTS isoforms...
  29. ncbi The complex life of WT1
    Kay Dietrich Wagner
    Johannes Müller Institut für Physiologie, Medizinische Fakultät Charité, Humboldt Universitat Berlin, Germany
    J Cell Sci 116:1653-8. 2003
    The Wilms' tumour gene, WT1, encodes a zinc-finger transcription factor that is inactivated in a subset of Wilms' tumours...
  30. ncbi WT1 is a modifier of the Pax2 mutant phenotype: cooperation and interaction between WT1 and Pax2
    Maria Teresa Discenza
    Department of Biochemistry, McGill University, Montreal, Quebec, Canada
    Oncogene 22:8145-55. 2003
    ..between the ureteric bud and progenitor mesenchyme, where the early expression of two genes, Wilms' tumour 1 (WT1) and paired box 2 (Pax2), establishes critical but unknown developmental pathways...
  31. pmc BASP1 is a transcriptional cosuppressor for the Wilms' tumor suppressor protein WT1
    Brian Carpenter
    School of Biological Sciences, University of Manchester, G 186 Stopford Building, Oxford Road, Manchester M13 9PT, UK
    Mol Cell Biol 24:537-49. 2004
    The Wilms' tumor suppressor protein WT1 is a transcriptional regulator that plays a key role in the development of the kidneys...
  32. ncbi Dysregulation of ferroportin 1 interferes with spleen organogenesis in polycythaemia mice
    Henry Mok
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Development 131:4871-81. 2004
    ..Thus, aberrant Fpn1 regulation and iron homeostasis interferes with development of the spleen stroma during embryogenesis, resulting in a novel defect in spleen architecture postnatally...
  33. ncbi The major podocyte protein nephrin is transcriptionally activated by the Wilms' tumor suppressor WT1
    Nicole Wagner
    Inserm U636, Centre de Biochimie, Parc Valrose, 06108 Nice, France
    J Am Soc Nephrol 15:3044-51. 2004
    ..This study analyzed a potential regulation of nephrin by the Wilms' tumor protein, Wt1. Using an inducible U2OS osteosarcoma cell line, it is shown that upon Wt1 induction, endogenous nephrin mRNA ..
  34. ncbi Odd-skipped related 1 is required for development of the metanephric kidney and regulates formation and differentiation of kidney precursor cells
    Richard G James
    Molecular and Vascular Medicine Unit, Beth Israel Deaconess Medical Center, 330 Brookline Ave, RW 663, Boston, MA 02215, USA
    Development 133:2995-3004. 2006
    ..These data indicate that Odd1 plays an important role in establishing kidney precursor cells, and in regulating their differentiation into kidney tubular tissue...
  35. ncbi The Wilms tumour suppressor protein WT1 (+KTS isoform) binds alpha-actinin 1 mRNA via its zinc-finger domain
    A A Morrison
    Bristol Genomics Research Institute, Faculty of Applied Sciences, University of the West of England, Coldharbour Lane, Bristol BS16 1QY, UK
    Biochem Cell Biol 84:789-98. 2006
    Mutations in WT1 are associated with developmental syndromes that affect the urogenital system and neoplasms, including Wilms tumour, acute myeloid leukemia, and breast and prostate cancers...
  36. pmc Discs-large homolog 1 regulates smooth muscle orientation in the mouse ureter
    Zhen X Mahoney
    Department of Internal Medicine, Renal Division, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 103:19872-7. 2006
    ..Our results suggest that (i) besides its well documented role in regulating epithelial polarity, Dlgh1 also regulates smooth muscle orientation, and (ii) human DLG1 mutations may contribute to hereditary forms of hydronephrosis...
  37. ncbi Adrenal development is initiated by Cited2 and Wt1 through modulation of Sf-1 dosage
    Pierre Val
    Section of Gene Function and Regulation, Institute of Cancer Research, 237 Fulham Road, London SW3 6JB, UK
    Development 134:2349-58. 2007
    ..We present genetic and molecular evidence demonstrating that Cited2 interacts with the transcription factor Wt1 to stimulate expression of the nuclear hormone receptor Sf-1 (Nr5a1) in the AGP prior to the separation between ..
  38. pmc The regulation of Sox9 gene expression by the GATA4/FOG2 transcriptional complex in dominant XX sex reversal mouse models
    Nikolay L Manuylov
    Department of Genetics, Dartmouth Medical School, Hanover, NH, USA
    Dev Biol 307:356-67. 2007
    ..Others have shown that XX mice with the Ods transgenic insertion or the Wt1-Sox9 YAC transgene overexpress the testis differentiation gene, Sox9...
  39. ncbi Upregulation of soluble vascular endothelial growth factor receptor 1 contributes to angiogenesis defects in the placenta of alpha 2B-adrenoceptor deficient mice
    Verena Muthig
    Institute of Experimental and Clinical Pharmacology, University of Freiburg, Albertstrasse 25, 79104 Freiburg, Germany
    Circ Res 101:682-91. 2007
    ....
  40. ncbi Expression of the Wilms' tumor gene WT1 in the murine urogenital system
    J Pelletier
    Center for Cancer Research, Massachusetts Institute of Technology, Cambridge 02139
    Genes Dev 5:1345-56. 1991
    The Wilms' tumor gene WT1 is a recessive oncogene that encodes a putative transcription factor implicated in nephrogenesis during kidney development. In this report we analyze expression of WT1 in the murine urogenital system...
  41. ncbi BMP-7 is an inducer of nephrogenesis, and is also required for eye development and skeletal patterning
    G Luo
    Department of Molecular Genetics, University of Texas M D Anderson Cancer Center, Houston 77030, USA
    Genes Dev 9:2808-20. 1995
    ..In addition, BMP-7-deficient mice have eye defects that appear to originate during lens induction. Finally, BMP-7-deficient mice also have skeletal patterning defects restricted to the rib cage, the skull, and the hindlimbs...
  42. ncbi Subnuclear localization of WT1 in splicing or transcription factor domains is regulated by alternative splicing
    S H Larsson
    Medical Research Council Human Genetics Unit, Western General Hospital, Edinburgh, Scotland
    Cell 81:391-401. 1995
    b>WT1 is a tumor suppressor gene with a key role in urogenital development and the pathogenesis of Wilms' tumor. Two alternative splice sites in the WT1 transcript allow the gene to encode four proteins...
  43. ncbi Expression of the Wilms' tumor suppressor gene WT1 during mouse embryogenesis
    R R Rackley
    Department of Cancer Biology, Cleveland Clinic Foundation, Ohio 44195 5001
    Cell Growth Differ 4:1023-31. 1993
    b>WT1 is a Wilms' tumor suppressor gene that maps to human chromosome 11p13 and encodes a putative transcription factor implicated in controlling normal urogenital development...
  44. ncbi The expression of the Wilms' tumour gene, WT1, in the developing mammalian embryo
    J F Armstrong
    MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK
    Mech Dev 40:85-97. 1993
    In the developing mouse, the Wilms' tumour gene, WT1, is first expressed in the intermediate mesenchyme lateral to the coelomic cavity (13 somite, early 9 dpc embryo)...
  45. ncbi The WT1 Wilms' tumor suppressor gene: how much do we really know?
    J C Reddy
    Brookdale Center for Molecular Biology, Mount Sinai School of Medicine, New York, NY, USA
    Biochim Biophys Acta 1287:1-28. 1996
  46. ncbi Defects of urogenital development in mice lacking Emx2
    N Miyamoto
    Department of Morphogenesis, Institute of Molecular Embryology and Genetics IMEG, Kumamoto University School of Medicine, Honjo, Japan
    Development 124:1653-64. 1997
    ..Degeneration of the Wolffian duct and mesonephric tubules was also abnormally accelerated without the formation of the Mullerian duct...
  47. ncbi Stromal cells mediate retinoid-dependent functions essential for renal development
    C Mendelsohn
    Columbia University, Department of Urology, New York, NY 10032, USA
    Development 126:1139-48. 1999
    ..Since c-ret signaling is required for ureteric bud morphogenesis, loss of c-ret expression is a likely cause of impaired ureteric bud branching in RARalpha beta2(-) mutants...
  48. ncbi YAC complementation shows a requirement for Wt1 in the development of epicardium, adrenal gland and throughout nephrogenesis
    A W Moore
    MRC Human Genetics Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK
    Development 126:1845-57. 1999
    The Wilms' Tumour gene WT1 has important functions during development. Knock-out mice were shown to have defects in the urogenital system and to die at embryonic day E13.5, probably due to heart failure...
  49. ncbi The Wilms tumor suppressor gene wt1 is required for development of the spleen
    U Herzer
    Research Center Karlsruhe Institute for Genetics P O Box 3640, 76021, Karlsruhe, Germany
    Curr Biol 9:837-40. 1999
    The Wilms tumor suppressor gene WT1 (wt1 in mouse) is unique among tumor suppressors because, in addition to its involvement in cancer [1] [2] and various other diseases [3] [4] [5] [6], it has an essential role in the development of ..
  50. ncbi Kidney development in cadherin-6 mutants: delayed mesenchyme-to-epithelial conversion and loss of nephrons
    S P Mah
    Molecular Neurobiology Laboratory, The Salk Institute for Biological Studies, La Jolla, California, 92037, USA
    Dev Biol 223:38-53. 2000
    ..These studies support the idea that cadherins play an essential role in the formation of epithelial structures and underscore the importance of timing in orchestrating the morphogenesis of complex epithelial tissues...
  51. ncbi FOG-2, a cofactor for GATA transcription factors, is essential for heart morphogenesis and development of coronary vessels from epicardium
    S G Tevosian
    Division of Hematology Oncology, Children s Hospital and Dana Farber Cancer Institute, Boston, Massachusetts 02115, USA
    Cell 101:729-39. 2000
    ..Our findings provide the molecular inroad into the induction of coronary vasculature by myocardium in the developing heart...
  52. ncbi Hoxa11 and Hoxd11 regulate branching morphogenesis of the ureteric bud in the developing kidney
    L T Patterson
    Division of Nephrology and Hypertension, The Children s Hospital Research Foundation, Cincinnati, OH 45229, USA
    Development 128:2153-61. 2001
    ....
  53. pmc The Wilms tumor suppressor WT1 regulates early gonad development by activation of Sf1
    Dagmar Wilhelm
    Institut für Toxikologie und Genetik, Forschungszentrum Karlsruhe, 76021 Karlsruhe, Germany
    Genes Dev 16:1839-51. 2002
    In mammals, several genes including the Wilms tumor suppressor gene Wt1, the Lim homeobox gene Lhx9, and the gene encoding steroidogenic factor 1 (Sf1) have been implicated in the development of the indifferent gonad prior to sexual ..
  54. ncbi Gonadal differentiation, sex determination and normal Sry expression in mice require direct interaction between transcription partners GATA4 and FOG2
    Sergei G Tevosian
    Division of Hematology and Oncology, Children s Hospital, Harvard Medical School, Boston, MA, USA
    Development 129:4627-34. 2002
    ..By contrast, Wt1 and Sf1, which are expressed prior to Sry and necessary for gonad development in both sexes, were expressed ..
  55. ncbi Pbx1 regulates nephrogenesis and ureteric branching in the developing kidney
    Catherine A Schnabel
    Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305 5342, USA
    Dev Biol 254:262-76. 2003
    ..Taken together, these data establish a role for Pbx1 in mesenchymal-epithelial signaling and demonstrate that Pbx1 is an essential regulator of mesenchymal function during renal morphogenesis...
  56. ncbi Regulation of metanephric kidney development by growth/differentiation factor 11
    Aurora F Esquela
    Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Dev Biol 257:356-70. 2003
    ..Our studies suggest that Gdf11 may be important in directing the initial outgrowth of the ureteric bud from the Wolffian duct by controlling the expression of Gdnf in the metanephric mesenchyme...
  57. 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
    ..These analyses establish a role for Six1 in the initial inductive step for metanephric development...
  58. ncbi The receptor tyrosine kinase regulator Sprouty1 is a target of the tumor suppressor WT1 and important for kidney development
    Isabelle Gross
    Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA
    J Biol Chem 278:41420-30. 2003
    b>WT1 encodes a transcription factor involved in kidney development and tumorigenesis...
  59. ncbi The wt1-heterozygous mouse; a model to study the development of glomerular sclerosis
    Aswin L Menke
    MRC Human Genetics Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, Scotland, UK
    J Pathol 200:667-74. 2003
    In the present study, it is shown that mice heterozygous for wt1 develop glomerular sclerosis and the nature and time course of events leading to the glomerular scarring are determined...
  60. ncbi Morphometric index of the developing murine kidney
    Cristina Cebrian
    Department of Physiology, Biophysics, and Systems Biology, Weill Medical College of Cornell University, 1300 York Avenue, New York, NY 10021, USA
    Dev Dyn 231:601-8. 2004
    ..In addition, this morphometric index provides a much-needed reference for accurately describing renal patterning defects exhibited by genetically altered mice...
  61. pmc The Wt1+/R394W mouse displays glomerulosclerosis and early-onset renal failure characteristic of human Denys-Drash syndrome
    Fei Gao
    Department of Molecular Genetics Cancer Genetics, Box 011, University of Texas M D Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77025, USA
    Mol Cell Biol 24:9899-910. 2004
    ..To help elucidate this, we generated a mouse strain carrying the missense mutation Wt1 R394W, which predisposes humans to glomerulosclerosis and early-onset renal failure (Denys-Drash syndrome [DDS])...
  62. ncbi Wnt9b plays a central role in the regulation of mesenchymal to epithelial transitions underlying organogenesis of the mammalian urogenital system
    Thomas J Carroll
    Department of Molecular and Cellular Biology, Harvard University, 16 Divinity Avenue, Cambridge, MA 02138, USA
    Dev Cell 9:283-92. 2005
    ..Together these findings suggest that Wnt9b is a common organizing signal regulating diverse components of the mammalian urogenital system...
  63. pmc Odd-skipped related 1 (Odd 1) is an essential regulator of heart and urogenital development
    Qingru Wang
    Center for Oral Biology and Department of Biomedical Genetics, University of Rochester School of Medicine and Dentistry, 601 Elmwood Avenue, Box 611, Rochester, NY 14642, USA
    Dev Biol 288:582-94. 2005
    ..marker analyses show that key regulators of early intermediate mesoderm development, including Lhx1, Pax2, and Wt1, are all down-regulated and nephrogenic mesenchyme undergoes massive apoptosis, resulting in disruption of nephric ..
  64. pmc Coronary vessel development requires activation of the TrkB neurotrophin receptor by the Wilms' tumor transcription factor Wt1
    Nicole Wagner
    Inserm U636, Centre de Biochimie, Faculte des Sciences, 06108 Nice, France
    Genes Dev 19:2631-42. 2005
    ..We report here expression of the Wilms' tumor gene-1, Wt1, in coronary vessels, which could contribute to the defective cardiac vascularization in Wt1-/- mice...
  65. pmc Tissue-specific RNAi reveals that WT1 expression in nurse cells controls germ cell survival and spermatogenesis
    Manjeet K Rao
    Department of Immunology, The University of Texas M D Anderson Cancer Center, Houston, Texas 77030, USA
    Genes Dev 20:147-52. 2006
    ..the principle by which naturally occurring microRNAs (miRNA) are made, we demonstrate that the Wilms' tumor 1 (WT1) transcription factor has an essential role in spermatogenesis...
  66. ncbi Wilms tumor suppressor, Wt1, is a transcriptional activator of the erythropoietin gene
    Christof Dame
    Department of Neonatology, Campus Virchow Klinikum, Charite Universitatsmedizin Berlin, Augustenburger Platz 1, D 13353 Berlin, Germany
    Blood 107:4282-90. 2006
    ..Recent findings indicate a role of the Wilms tumor suppressor, Wt1, in the formation of the hematopoietic system...
  67. ncbi Male-biased expression of X-chromosomal DM domain-less Dmrt8 genes in the mouse
    Anne Marie Veith
    Department of Physiological Chemistry I, Biozentrum, University of Wurzburg, Am Hubland, D 97074 Wurzburg, Germany
    Genomics 88:185-95. 2006
    ..Dmrt8.1 mRNA was detected in Sertoli cells by in situ hybridization. In embryos, Dmrt8.2 shows a dynamic expression restricted to male and female gonads and might therefore be involved in sexual development in the mouse...
  68. pmc Tbx18 regulates the development of the ureteral mesenchyme
    Rannar Airik
    Institut fur Molekularbiologie, Medizinische Hochschule Hannover, Hannover, Germany
    J Clin Invest 116:663-74. 2006
    ..Our analysis also showed that the ureteral mesenchyme derives from a distinct cell population that is separated early in kidney development from that of other mesenchymal cells of the renal system...
  69. pmc The Wilms' tumor 1 (WT1) gene (+KTS isoform) functions with a CTE to enhance translation from an unspliced RNA with a retained intron
    Yeou Cherng Bor
    Myles H Thaler Center for AIDS and Human Retrovirus Research and Department of Microbiology, University of Virginia, Charlottesville, Virginia 22908, USA
    Genes Dev 20:1597-608. 2006
    The Wilms' tumor 1 (WT1) gene plays an important role in mammalian urogenital development, and dysregulation of this gene is observed in many human cancers...
  70. pmc The Wilms tumor gene, Wt1, is required for Sox9 expression and maintenance of tubular architecture in the developing testis
    Fei Gao
    Department of Molecular Genetics Cancer Genetics, University of Texas M D Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA
    Proc Natl Acad Sci U S A 103:11987-92. 2006
    Mutation of the transcription factor and tumor suppressor gene WT1 results in a range of genitourinary anomalies in humans, including 46,XY gonadal dysgenesis, indicating that WT1 plays a critical role in sex determination...
  71. pmc Notch2, but not Notch1, is required for proximal fate acquisition in the mammalian nephron
    Hui Teng Cheng
    Department of Molecular Biology and Pharmacology at Washington University School of Medicine, 660 South Euclid Avenue, Campus Box 8103, St Louis, MO 63110, USA
    Development 134:801-11. 2007
    ..These results establish distinct (non-redundant), instructive roles for Notch receptors in nephron segmentation...
  72. ncbi The Wilms' tumor gene WT1-GFP knock-in mouse reveals the dynamic regulation of WT1 expression in normal and leukemic hematopoiesis
    N Hosen
    Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine, Stanford, CA, USA
    Leukemia 21:1783-91. 2007
    The Wilms' tumor gene WT1 is overexpressed in most of human leukemias regardless of disease subtypes...
  73. pmc The ECM protein nephronectin promotes kidney development via integrin alpha8beta1-mediated stimulation of Gdnf expression
    James M Linton
    Department of Physiology, 1550 Fourth Street, University of California, San Francisco, San Francisco, CA 94143, USA
    Development 134:2501-9. 2007
    ..Our results thus place nephronectin and alpha8beta1 integrin in a pathway that regulates Gdnf expression and is essential for kidney development...
  74. pmc Alternately spliced WT1 antisense transcripts interact with WT1 sense RNA and show epigenetic and splicing defects in cancer
    Anthony R Dallosso
    CLIC Sargent Research Unit, Department of Cellular and Molecular Medicine, School of Medical Sciences, University of Bristol, Bristol BS8 1TD, United Kingdom
    RNA 13:2287-99. 2007
    ..b>WT1 is a well-characterized developmental gene that is mutated in Wilms' tumor (WT) and acute myeloid leukaemia (AML) ..
  75. pmc A GATA4/WT1 cooperation regulates transcription of genes required for mammalian sex determination and differentiation
    Yoko Miyamoto
    Ontogeny Reproduction Research Unit, Centre de Recherche du Centre Hospitalier Universitaire de Quebec CRCHUQ, Quebec City, QC, G1V 4G2, Canada
    BMC Mol Biol 9:44. 2008
    ....
  76. doi A myocardial lineage derives from Tbx18 epicardial cells
    Chen Leng Cai
    Skaggs School of Pharmacy, University of California, San Diego, La Jolla, California 92093, USA
    Nature 454:104-8. 2008
    ..The pluripotency of Tbx18 proepicardial cells provides a theoretical framework for applying these progenitors to effect cardiac repair and regeneration...
  77. pmc Epicardial progenitors contribute to the cardiomyocyte lineage in the developing heart
    Bin Zhou
    Harvard Stem Cell Institute and Department of Cardiology, Children s Hospital Boston, 300 Longwood Avenue, Boston, Massachusetts 02115, USA
    Nature 454:109-13. 2008
    ..Here we identify a novel cardiogenic precursor marked by expression of the transcription factor Wt1 and located within the epicardium-an epithelial sheet overlying the heart...
  78. pmc Nkx2-5- and Isl1-expressing cardiac progenitors contribute to proepicardium
    Bin Zhou
    Harvard Stem Cell Institute and Department of Cardiology, Children s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Biochem Biophys Res Commun 375:450-3. 2008
    ..Both Nkx2-5- and Isl1-expressing progenitors contributed to the proepicardium and expressed Wt1 and Tbx18, markers of proepicardial progenitor cells...
  79. pmc Hedgehog signaling to distinct cell types differentially regulates coronary artery and vein development
    Kory J Lavine
    Department of Developmental Biology, Washington University School of Medicine, St Louis, MO 63110, USA
    Development 135:3161-71. 2008
    ..Finally, we present evidence suggesting that coronary arteries and veins may be derived from distinct lineages...
  80. pmc Mesothelium contributes to vascular smooth muscle and mesenchyme during lung development
    Jianwen Que
    Department of Cell Biology, Duke University Medical Center, Durham, NC 27710, USA
    Proc Natl Acad Sci U S A 105:16626-30. 2008
    ..In the developing lung, the Wilm's tumor 1 gene (Wt1) is expressed only in the mesothelial cells...
  81. doi Tbx18 and the fate of epicardial progenitors
    Vincent M Christoffels
    Department of Anatomy and Embryology, Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Meibergdreef 15 L2 108, 1105 AZ Amsterdam, The Netherlands
    Nature 458:E8-9; discussion E9-10. 2009
    ....
  82. pmc GLI3 repressor controls nephron number via regulation of Wnt11 and Ret in ureteric tip cells
    Jason E Cain
    Program in Developmental and Stem Cell Biology, The Hospital for Sick Children, Toronto Medical Discovery Towers, Toronto, Ontario, Canada
    PLoS ONE 4:e7313. 2009
    ..Thus, GLI3 repressor controls nephron number by regulating ureteric tip cell expression of Wnt11 and Ret...
  83. pmc Genetic fate mapping demonstrates contribution of epicardium-derived cells to the annulus fibrosis of the mammalian heart
    Bin Zhou
    Department of Cardiology, Children s Hospital Boston and Department of Genetics, Harvard Medical School, Boston, MA 02115, USA
    Dev Biol 338:251-61. 2010
    ..Abnormalities of this differentiation process may underlie development of some forms of re-entrant atrioventricular tachycardia...
  84. pmc Wt1 and retinoic acid signaling in the subcoelomic mesenchyme control the development of the pleuropericardial membranes and the sinus horns
    Julia Norden
    Institut fur Molekularbiologie, OE5250, Medizinische Hochschule Hannover, Carl Neuberg Str 1, D 30625 Hannover, Germany
    Circ Res 106:1212-20. 2010
    ..The systemic venous return myocardium (sinus node and sinus horns) forms only late in cardiogenesis from a pool of pericardial mesenchymal precursor cells...
  85. pmc Coronary arteries form by developmental reprogramming of venous cells
    Kristy Red-Horse
    Department of Biochemistry and Howard Hughes Medical Institute, Stanford, California 94305 5307, USA
    Nature 464:549-53. 2010
    ..Understanding this new reprogramming process and identifying the endogenous signals should suggest more natural ways of engineering coronary bypass grafts and revascularizing the heart...
  86. pmc Regulation of embryonic kidney branching morphogenesis and glomerular development by KISS1 receptor (Gpr54) through NFAT2- and Sp1-mediated Bmp7 expression
    Tingfang Yi
    Department of Molecular and Cellular Medicine, Texas A and M University Health Science Center, Institute of BioScience and Technology, Center for Cancer and Stem Cell Biology, Houston, Texas 77030, USA
    J Biol Chem 285:17811-20. 2010
    ..Together, these data suggest that Gpr54 regulates Bmp7 expression through NFAT2 and Sp1 and plays an important role in embryonic kidney branching morphogenesis and glomerular development...
  87. pmc Dicer regulates the development of nephrogenic and ureteric compartments in the mammalian kidney
    Vidya K Nagalakshmi
    Department of Cell Biology, University of Virginia School of Medicine, Charlottesville, Virginia 22908, USA
    Kidney Int 79:317-30. 2011
    ..Furthermore, an understanding of miRNA action may provide new insights into the etiology and pathogenesis of renal cyst-based kidney disease...
  88. pmc Septum transversum-derived mesothelium gives rise to hepatic stellate cells and perivascular mesenchymal cells in developing mouse liver
    Kinji Asahina
    Southern California Research Center for ALPD and Cirrhosis and Department of Pathology, Keck School of Medicine of the University of Southern California, Los Angeles, CA
    Hepatology 53:983-95. 2011
    ..5 share the expression of activated leukocyte cell adhesion molecule (Alcam), desmin, and Wilms tumor 1 homolog (Wt1)...
  89. pmc Conditional ablation of Gata4 and Fog2 genes in mice reveals their distinct roles in mammalian sexual differentiation
    N L Manuylov
    Department of Genetics, Dartmouth Medical School, Hanover, NH 03755, USA
    Dev Biol 353:229-41. 2011
    ..Our results now demonstrate that these two genes also have non-overlapping essential functions in testis development...
  90. pmc Tight regulation of p53 activity by Mdm2 is required for ureteric bud growth and branching
    Sylvia Hilliard
    Tulane University School of Medicine, Department of Pediatrics, Section of Pediatric Nephrology, Orleans, LA 70112, USA
    Dev Biol 353:354-66. 2011
    ..These results demonstrate a critical and cell autonomous role for Mdm2 in the UB lineage. Mdm2-mediated inhibition of p53 activity is a prerequisite for renal organogenesis...
  91. pmc WT1 regulates epicardial epithelial to mesenchymal transition through β-catenin and retinoic acid signaling pathways
    Alexander von Gise
    Department of Cardiology, Children s Hospital Boston, 300 Longwood Ave, Boston, MA, USA
    Dev Biol 356:421-31. 2011
    ..In the developing embryo, the epicardium expresses the transcriptional regulator Wilm's Tumor Gene 1 (Wt1). Through incompletely understood mechanisms, Wt1 inactivation derails normal heart development...
  92. pmc Developmental patterns and characteristics of epicardial cell markers Tbx18 and Wt1 in murine embryonic heart
    Bin Zeng
    Department of Cardiology, Renmin Hospital of Wuhan University, Wuhan, Hubei, PR China
    J Biomed Sci 18:67. 2011
    ..The present study therefore aimed to examine the the expression of Tbx18 and Wt1 in embryonic heart and to identify whether Tbx18 and Wt1 themselves expressed in the cardiomyocyte.
  93. pmc A wt1-controlled chromatin switching mechanism underpins tissue-specific wnt4 activation and repression
    Abdelkader Essafi
    MRC Human Genetics Unit and Institute for Genetics and Molecular Medicine, Western General Hospital, Edinburgh EH4 2XU, UK
    Dev Cell 21:559-74. 2011
    b>Wt1 regulates the epithelial-mesenchymal transition (EMT) in the epicardium and the reverse process (MET) in kidney mesenchyme. The mechanisms underlying these reciprocal functions are unknown...
  94. pmc Epicardially derived fibroblasts preferentially contribute to the parietal leaflets of the atrioventricular valves in the murine heart
    Andy Wessels
    Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, USA
    Dev Biol 366:111-24. 2012
    ....
  95. doi Wt1 and epicardial fate mapping
    Carsten Rudat
    Institut fur Molekularbiologie, Medizinische Hochschule Hannover, Hannover, Germany
    Circ Res 111:165-9. 2012
    ..Genetic lineage tracing systems based on Wt1 regulatory sequences provided evidence that epicardium-derived cells also adopt a myocardial fate in the mouse.
  96. doi Tbx18 function in epicardial development
    Franziska Greulich
    Institut fur Molekularbiologie, Medizinische Hochschule Hannover, OE5250, Carl Neuberg Str 1, Hannover D 30625, Germany
    Cardiovasc Res 96:476-83. 2012
    ..We aimed to elucidate the functional significance of the conserved epicardial expression of the T-box transcription factor gene Tbx18 using transgenic technology in the mouse...
  97. doi Wt1-expressing progenitors contribute to multiple tissues in the developing lung
    Elena Cano
    Dept of Animal Biology, Faculty of Sciences, Univ of Malaga, 29071 Malaga, Spain
    Am J Physiol Lung Cell Mol Physiol 305:L322-32. 2013
    ..In the developing lungs, Wt1, the Wilms' tumor suppressor gene, is specifically expressed in the embryonic mesothelium...
  98. pmc Gata4 is required for formation of the genital ridge in mice
    Yueh Chiang Hu
    Whitehead Institute, Howard Hughes Medical Institute, and Department of Biology, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA
    PLoS Genet 9:e1003629. 2013
    ..At least four genes (Lhx9, Sf1, Wt1, and Emx2) have been demonstrated to be required for subsequent growth and maintenance of the genital ridge...
  99. pmc Hox11 paralogous genes are essential for metanephric kidney induction
    Deneen M Wellik
    Department of Human Genetics and Howard Hughes Medical Institute, University of Utah School of Medicine, Salt Lake City 84112 5331, USA
    Genes Dev 16:1423-32. 2002
    ..In these triple mutants, the metanephric blastema condenses, and expression of early patterning genes, Pax2 and Wt1, is unperturbed. Eya1 expression is also intact...
  100. ncbi Murine Denys-Drash syndrome: evidence of podocyte de-differentiation and systemic mediation of glomerulosclerosis
    Charles E Patek
    Molecular Medicine Centre, Western General Hospital, Edinburgh, UK
    Hum Mol Genet 12:2379-94. 2003
    Denys-Drash syndrome (DDS) is caused by dominant mutations of the Wilms' tumour suppressor gene, WT1, and characterized by a nephropathy involving diffuse mesangial sclerosis, male pseudohermaphroditism and/or Wilms' tumourigenesis...
  101. ncbi Pbx1 is essential for adrenal development and urogenital differentiation
    Catherine A Schnabel
    Genesis 37:123-30. 2003
    ..These findings establish a Pbx1-dependent pathway that regulates the expansion of SF-1 positive cells essential for adrenal formation and gonadal differentiation and demonstrate an early requirement for Pbx1 in urogenital development...

Research Grants4

  1. Pathogenic Mechanism of Polycystic Kidney Disease
    Yumay Chen; Fiscal Year: 2010
    ..Our studies will provide novel insight into the molecular pathogenesis of PKD. They will help explain why cysts progress at different rates and how therapies to slow the progression of PKD can be designed rationally. ..
  2. Transcription Factor P53 in Terminal Nephron Different
    Zubaida Saifudeen; Fiscal Year: 2004
    ..The results of this work will provide information on what determines terminal renal differentiation. In addition, these data will advance our understanding of the role the tumor suppressor p53 in kidney development...
  3. The Role of Cartilage Link Protein 1 (Crtl1) in Heart Development
    ARNO WESSELS; Fiscal Year: 2010
    ..Understanding the role of Crtl1, and Crtl1-associated mechanisms, in heart development may further our knowledge of the etiology of a variety of these malformations including the mechanisms that lead to muscular septal defects. ..
  4. Immunobiological Role of Fertilization Antigen(FA-1)
    RAJESH NAZ; Fiscal Year: 2007
    ..unreadable] [unreadable]..