The candidate Wilms' tumour gene is involved in genitourinary development

Kathryn Pritchard-Jones, Stewart Fleming, Duncan Davidson, Wendy Bickmore, David Porteous, Christine Gosden, Jonathan Bard, Alan Buckler, Jerry Pelletier, David Housman, Veronica van Heyningen, Nicholas Hastie

    Research output: Contribution to journalArticle

    714 Citations (Scopus)

    Abstract

    Wilms' tumour is an embryonic kidney tumour thought to arise through aberrant mesenchymal stem cell differentiation and to result from loss of function of a 'tumour suppressor' gene(s). Both sporadic and syndrome-associated Wilms' tumours are accompanied by an increased frequency of abnormalities of the urinary tract and genitalia. Deletional analysis of individuals with the WAGR syndrome (for, Wilms' tumour, aniridia, genitourinary abnormalities and mental retardation) showed that a Wilms' tumour gene lies at chromosomal position 11p13. This led to the isolation of a candidate Wilms' tumour gene, encoding a zinc-finger protein which is likely to be a transcription factor. To gain insight into the role of this candidate gene in normal development and tumorigenesis, we have now performed in situ messenger RNA hybridization on sections of human embryos and Wilms' tumours. The candidate Wilms' tumour gene is expressed specifically in the condensed mesenchyme, renal vesicle and glomerular epithelium of the developing kidney, in the related mesonephric glomeruli and in cells approximating these structures in tumours. The other main sites of expression are the genital ridge, fetal gonad and mesothelium. These data suggest that (1) this candidate is indeed a Wilms' tumour gene, (2) the associated genital abnormalities are pleiotropic effects of mutation in the Wilms' tumour gene itself, in support of recent genetic analysis, and (3) this gene has a specific role in kidney development and a wider role in mesenchymal-epithelial transitions.
    Original languageEnglish
    Pages (from-to)194-7
    Number of pages4
    JournalNature
    Volume346
    Issue number6280
    DOIs
    Publication statusPublished - 1990

    Fingerprint

    Wilms' Tumor Genes
    Wilms Tumor
    Kidney
    WAGR Syndrome
    Epithelium
    Urogenital Abnormalities
    Aniridia
    Genitalia
    Epithelial-Mesenchymal Transition
    Zinc Fingers
    Gonads
    Mesoderm
    Tumor Suppressor Genes
    Urinary Tract
    Mesenchymal Stromal Cells
    Intellectual Disability
    Genes
    Cell Differentiation
    Neoplasms
    Carcinogenesis

    Cite this

    Pritchard-Jones, K., Fleming, S., Davidson, D., Bickmore, W., Porteous, D., Gosden, C., ... Hastie, N. (1990). The candidate Wilms' tumour gene is involved in genitourinary development. Nature, 346(6280), 194-7. https://doi.org/10.1038/346194a0
    Pritchard-Jones, Kathryn ; Fleming, Stewart ; Davidson, Duncan ; Bickmore, Wendy ; Porteous, David ; Gosden, Christine ; Bard, Jonathan ; Buckler, Alan ; Pelletier, Jerry ; Housman, David ; van Heyningen, Veronica ; Hastie, Nicholas. / The candidate Wilms' tumour gene is involved in genitourinary development. In: Nature. 1990 ; Vol. 346, No. 6280. pp. 194-7.
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    abstract = "Wilms' tumour is an embryonic kidney tumour thought to arise through aberrant mesenchymal stem cell differentiation and to result from loss of function of a 'tumour suppressor' gene(s). Both sporadic and syndrome-associated Wilms' tumours are accompanied by an increased frequency of abnormalities of the urinary tract and genitalia. Deletional analysis of individuals with the WAGR syndrome (for, Wilms' tumour, aniridia, genitourinary abnormalities and mental retardation) showed that a Wilms' tumour gene lies at chromosomal position 11p13. This led to the isolation of a candidate Wilms' tumour gene, encoding a zinc-finger protein which is likely to be a transcription factor. To gain insight into the role of this candidate gene in normal development and tumorigenesis, we have now performed in situ messenger RNA hybridization on sections of human embryos and Wilms' tumours. The candidate Wilms' tumour gene is expressed specifically in the condensed mesenchyme, renal vesicle and glomerular epithelium of the developing kidney, in the related mesonephric glomeruli and in cells approximating these structures in tumours. The other main sites of expression are the genital ridge, fetal gonad and mesothelium. These data suggest that (1) this candidate is indeed a Wilms' tumour gene, (2) the associated genital abnormalities are pleiotropic effects of mutation in the Wilms' tumour gene itself, in support of recent genetic analysis, and (3) this gene has a specific role in kidney development and a wider role in mesenchymal-epithelial transitions.",
    author = "Kathryn Pritchard-Jones and Stewart Fleming and Duncan Davidson and Wendy Bickmore and David Porteous and Christine Gosden and Jonathan Bard and Alan Buckler and Jerry Pelletier and David Housman and {van Heyningen}, Veronica and Nicholas Hastie",
    year = "1990",
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    Pritchard-Jones, K, Fleming, S, Davidson, D, Bickmore, W, Porteous, D, Gosden, C, Bard, J, Buckler, A, Pelletier, J, Housman, D, van Heyningen, V & Hastie, N 1990, 'The candidate Wilms' tumour gene is involved in genitourinary development', Nature, vol. 346, no. 6280, pp. 194-7. https://doi.org/10.1038/346194a0

    The candidate Wilms' tumour gene is involved in genitourinary development. / Pritchard-Jones, Kathryn; Fleming, Stewart; Davidson, Duncan; Bickmore, Wendy ; Porteous, David ; Gosden, Christine; Bard, Jonathan; Buckler, Alan; Pelletier, Jerry; Housman, David; van Heyningen, Veronica; Hastie, Nicholas.

    In: Nature, Vol. 346, No. 6280, 1990, p. 194-7.

    Research output: Contribution to journalArticle

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    T1 - The candidate Wilms' tumour gene is involved in genitourinary development

    AU - Pritchard-Jones, Kathryn

    AU - Fleming, Stewart

    AU - Davidson, Duncan

    AU - Bickmore, Wendy

    AU - Porteous, David

    AU - Gosden, Christine

    AU - Bard, Jonathan

    AU - Buckler, Alan

    AU - Pelletier, Jerry

    AU - Housman, David

    AU - van Heyningen, Veronica

    AU - Hastie, Nicholas

    PY - 1990

    Y1 - 1990

    N2 - Wilms' tumour is an embryonic kidney tumour thought to arise through aberrant mesenchymal stem cell differentiation and to result from loss of function of a 'tumour suppressor' gene(s). Both sporadic and syndrome-associated Wilms' tumours are accompanied by an increased frequency of abnormalities of the urinary tract and genitalia. Deletional analysis of individuals with the WAGR syndrome (for, Wilms' tumour, aniridia, genitourinary abnormalities and mental retardation) showed that a Wilms' tumour gene lies at chromosomal position 11p13. This led to the isolation of a candidate Wilms' tumour gene, encoding a zinc-finger protein which is likely to be a transcription factor. To gain insight into the role of this candidate gene in normal development and tumorigenesis, we have now performed in situ messenger RNA hybridization on sections of human embryos and Wilms' tumours. The candidate Wilms' tumour gene is expressed specifically in the condensed mesenchyme, renal vesicle and glomerular epithelium of the developing kidney, in the related mesonephric glomeruli and in cells approximating these structures in tumours. The other main sites of expression are the genital ridge, fetal gonad and mesothelium. These data suggest that (1) this candidate is indeed a Wilms' tumour gene, (2) the associated genital abnormalities are pleiotropic effects of mutation in the Wilms' tumour gene itself, in support of recent genetic analysis, and (3) this gene has a specific role in kidney development and a wider role in mesenchymal-epithelial transitions.

    AB - Wilms' tumour is an embryonic kidney tumour thought to arise through aberrant mesenchymal stem cell differentiation and to result from loss of function of a 'tumour suppressor' gene(s). Both sporadic and syndrome-associated Wilms' tumours are accompanied by an increased frequency of abnormalities of the urinary tract and genitalia. Deletional analysis of individuals with the WAGR syndrome (for, Wilms' tumour, aniridia, genitourinary abnormalities and mental retardation) showed that a Wilms' tumour gene lies at chromosomal position 11p13. This led to the isolation of a candidate Wilms' tumour gene, encoding a zinc-finger protein which is likely to be a transcription factor. To gain insight into the role of this candidate gene in normal development and tumorigenesis, we have now performed in situ messenger RNA hybridization on sections of human embryos and Wilms' tumours. The candidate Wilms' tumour gene is expressed specifically in the condensed mesenchyme, renal vesicle and glomerular epithelium of the developing kidney, in the related mesonephric glomeruli and in cells approximating these structures in tumours. The other main sites of expression are the genital ridge, fetal gonad and mesothelium. These data suggest that (1) this candidate is indeed a Wilms' tumour gene, (2) the associated genital abnormalities are pleiotropic effects of mutation in the Wilms' tumour gene itself, in support of recent genetic analysis, and (3) this gene has a specific role in kidney development and a wider role in mesenchymal-epithelial transitions.

    U2 - 10.1038/346194a0

    DO - 10.1038/346194a0

    M3 - Article

    C2 - 2164159

    VL - 346

    SP - 194

    EP - 197

    JO - Nature

    JF - Nature

    SN - 0028-0836

    IS - 6280

    ER -

    Pritchard-Jones K, Fleming S, Davidson D, Bickmore W, Porteous D, Gosden C et al. The candidate Wilms' tumour gene is involved in genitourinary development. Nature. 1990;346(6280):194-7. https://doi.org/10.1038/346194a0