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Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping

Nature volume 343, pages 774–778 (1990)Cite this article

Abstract

CYTOGENETIC analysis has identified chromosome Ilpl3 as the smallest overlap region for deletions found in individuals with WAGR syndrome, which includes Wilms tumour (a recessive childhood nephroblastoma), anirida, genito-urinary abnormalities and mental retardation1. The underlying loci have since been resolved into an aniridia (AN2) locus at a telomeric position, and a locus of closely spaced genes or a single pleiotropic gene involved in genito-urinary tract abnormalities and Wilms tumour at a more centromeric position2–7. Pulsed-field gel analysis of the 11pl3 region has revealed the presence of several putative CpG islands8,9, structures which are frequently associated with the 5' ends of expressed sequences, mainly housekeeping genes and some tissue-specific genes10. Starting from a CpG island, we have now isolated four neighbouring CpG islands, all within 650 kilobases (kb), by means of two consecutive bidirectional jumps in rare-cutting restriction-enzyme jumping libraries11. In two instances, flanking sequences were conserved in other species and RNA transcripts were identified. A complementary DNA clone isolated for one of them derives from an RNA highly expressed in fetal kidney, and is predicted to encode a Krüppel-like12 zinc-finger protein that is probably a transcription factor. The entire cDNA region is included in two partially overlapping homozygous deletions found in Wilms tumour DNA samples. Cloning of the breakpoints in one tumour revealed a deletion size of 170 kb, one-third of which is covered by the cDNA. The expression pattern and sequence of this cDNA could point to an important role for its corresponding gene in the normal development of the renal system as well as in Wilms tumour.

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References

  1. Francke, U., Holmes, L. B., Atkins, L. & Riccardi, V. M. Cytogenet. Cell Genet. 24, 185–192 (1979).

    Article  CAS  Google Scholar 

  2. Turleau, C. et al. Hum. Genet. 67, 455–456 (1984).

    Article  CAS  Google Scholar 

  3. Glaser, T. et al. Nature 321, 882–887 (1986).

    Article  ADS  CAS  Google Scholar 

  4. Porteous, D. et al. Proc. natn. Acad. Sci. U.S.A. 84, 5355–5359 (1987).

    Article  ADS  CAS  Google Scholar 

  5. Gessler, M. et al. Am. J. hum. Genet. 44, 486–495 (1989).

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Gessler, M. & Bruns, G. A. P. Cytogenet. Cell Genet. 51, 1003 (1989).

    Google Scholar 

  7. van Heyningen, V. et al. Cytogenet. Cell Genet. 51, 1095 (1989).

    Google Scholar 

  8. Compton, D. A. et al. Cell 55, 827–836 (1988).

    Article  CAS  Google Scholar 

  9. Gessler, M. & Bruns, G. A. P. Genomics 5, 43–55 (1989).

    Article  CAS  Google Scholar 

  10. Bird, A. P. Nature 321, 209–213 (1986).

    Article  ADS  CAS  Google Scholar 

  11. Poustka, A., Pohl, T. M., Barlow, D. P. & Frischauf, A.-M. Nature 325, 353–355 (1987).

    Article  ADS  CAS  Google Scholar 

  12. Rosenberg, U. et al. Nature 319, 336–339 (1986).

    Article  ADS  CAS  Google Scholar 

  13. Poustka, A. & Lehrach, H. Genet Engin. Vol. 10 (ed. Setlow, J. K.) 169–195 (Plenum Press, New York and London, 1988).

    Book  Google Scholar 

  14. Kozak, M. Nucleic. Acids Res. 15, 8125–8132 (1987).

    Article  CAS  Google Scholar 

  15. Chavier, P. et al. EMBO J. 7, 29–35 (1988).

    Article  Google Scholar 

  16. Joseph, L. J. et al. Proc. natn. Acad. Sci. U.S.A. 85, 7164–7168 (1988).

    Article  ADS  CAS  Google Scholar 

  17. Mermod, N., O'Neill, E. A., Kelly, T. J. & Tijan, R. Cell 58, 741–753 (1989).

    Article  CAS  Google Scholar 

  18. Bonetta, L., Huang, A., Gregoris, M., Yeger, H. & Williams, B. R. G. Cytogenet. Cell Genet. 51, 965 (1989).

    Google Scholar 

  19. Call, K. et al. Cytogenet. Cell Genet. 51, 974 (1989).

    Google Scholar 

  20. Friend, S. H. et al. Nature 323, 643–646 (1986).

    Article  ADS  CAS  Google Scholar 

  21. Fung, Y.-K. T. et al. Science 236, 1657–1661 (1987).

    Article  ADS  CAS  Google Scholar 

  22. Lee, W.-H. et al. Science 235, 1394–1399 (1987).

    Article  ADS  CAS  Google Scholar 

  23. Goddard, A. D. et al. Molec. cell. Biol. 8, 2082–2088 (1988).

    Article  CAS  Google Scholar 

  24. Harbour, J. W. et al. Science 241, 353–357 (1988).

    Article  ADS  CAS  Google Scholar 

  25. Grundy, P. et al. Nature 336, 374–376 (1988).

    Article  ADS  CAS  Google Scholar 

  26. Huff, V. et al. Nature 336, 377–378 (1988).

    Article  ADS  CAS  Google Scholar 

  27. Koufos, A. et al. Am. J. hum. Genet. 44, 711–719 (1989).

    CAS  PubMed  PubMed Central  Google Scholar 

  28. Henry, I. et al. Proc. natn. Acad. Sci. U.S.A. 86, 3247–3251 (1989).

    Article  ADS  CAS  Google Scholar 

  29. Reeve, A. E., Sih, S. A., Raizis, A. M. & Feinberg, A. P. Molec. cell. Biol. 9, 1799–1803 (1989).

    Article  CAS  Google Scholar 

  30. Herrmann, B. G., Barlow, D. P. & Lehrach, H. Cell 48, 813–825 (1987).

    Article  CAS  Google Scholar 

  31. Maniatis, T., Fritsch, E. F. & Sambrook, J. Molecular Cloning (Cold Spring Harbor Laboratory, Cold Spring Harbor, New York, 1982).

    Google Scholar 

  32. Schuh, R. et al. Cell 47, 1025–1032 (1986).

    Article  CAS  Google Scholar 

Download references

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Author notes

  1. Manfred Gessler

    Present address: Institut für Humangenetik, Philips-Universität, Marburg, FRG

  2. Annemarie Poustka: German Cancer Research Center, Heidelberg, FRG

  3. Webster Cavenee: Ludwig Institute for Cancer Research, Montreal, Canada

  4. Rachael L. Neve: Department of Psychobiology, University of California-Irvine, Irvine, California 92717, USA

  5. Stuart H. Orkin: Division of Hematology-Oncology, Children's Hospital and Dana-Farber Cancer Institute, Department of Pediatrics, Harvard Medical School, Howard Hughes Medical Institute, Boston, Massachusetts 02115, USA

Authors and Affiliations

  1. Genetics Division, The Children's Hospital, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, 02115, USA

    Manfred Gessler, Annemarie Poustka, Webster Cavenee, Rachael L. Neve, Stuart H. Orkin & Gail A. P. Bruns

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  6. Gail A. P. Bruns
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Gessler, M., Poustka, A., Cavenee, W. et al. Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping. Nature 343, 774–778 (1990). https://doi.org/10.1038/343774a0

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