Skip to main content
PMC home page
American Journal of Human Genetics logoLink to American Journal of Human Genetics
. 1990 Apr;46(4):789–794.

The ataxia-telangiectasia gene (ATA) on chromosome II is distinct from the ETS-1 gene.

P Concannon 1, U Malhotra 1, P Charmley 1, J Reynolds 1, K Lange 1, R A Gatti 1
PMCID: PMC1683674  PMID: 1969227

Abstract

We have studied the segregation of an RFLP detected with a human ETS-1 genomic probe in 25 families containing members affected with ataxia-telangiectasia (AT) and in 27 families from the Centre d'Etude du Polymorphisme Humain (CEPH) panel. We have recently mapped a gene for AT to 11q22-23 by linkage to the markers THY1 and D11S144. Multipoint linkage analysis of the CEPH families indicated that ETS-1 is located on chromosome 11q approximately 19.2 centimorgans telomeric to THY1. Analysis of the segregation of ETS-1 alleles in AT families yields strongly negative LOD scores, excluding an AT gene from a region extending 15 cM to either side of ETS-1. Multipoint mapping of ETS-1, D11S144, THY1, and AT also excludes the possibility that an AT gene is telomeric to ETS-1.

Full text

PDF
791

Images in this article

791Figure 1
on p.791

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Budarf M., Sellinger B., Griffin C., Emanuel B. S. Comparative mapping of the constitutional and tumor-associated 11;22 translocations. Am J Hum Genet. 1989 Jul;45(1):128–139. [PMC free article] [PubMed] [Google Scholar]
  2. Chen J. H. The proto-oncogene c-ets is preferentially expressed in lymphoid cells. Mol Cell Biol. 1985 Nov;5(11):2993–3000. doi: 10.1128/mcb.5.11.2993. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Curry C. J., O'Lague P., Tsai J., Hutchison H. T., Jaspers N. G., Wara D., Gatti R. A., Hutchinson H. T. ATFresno: a phenotype linking ataxia-telangiectasia with the Nijmegen breakage syndrome. Am J Hum Genet. 1989 Aug;45(2):270–275. [PMC free article] [PubMed] [Google Scholar]
  4. Dausset J. Le centre d'étude du polymorphisme humain. Presse Med. 1986 Oct 18;15(36):1801–1802. [PubMed] [Google Scholar]
  5. Diaz M. O., Le Beau M. M., Pitha P., Rowley J. D. Interferon and c-ets-1 genes in the translocation (9;11)(p22;q23) in human acute monocytic leukemia. Science. 1986 Jan 17;231(4735):265–267. doi: 10.1126/science.3455787. [DOI] [PubMed] [Google Scholar]
  6. Feinberg A. P., Vogelstein B. "A technique for radiolabeling DNA restriction endonuclease fragments to high specific activity". Addendum. Anal Biochem. 1984 Feb;137(1):266–267. doi: 10.1016/0003-2697(84)90381-6. [DOI] [PubMed] [Google Scholar]
  7. Fujiwara S., Fisher R. J., Seth A., Bhat N. K., Showalter S. D., Zweig M., Papas T. S. Characterization and localization of the products of the human homologs of the v-ets oncogene. Oncogene. 1988 Feb;2(2):99–103. [PubMed] [Google Scholar]
  8. Gatti R. A., Berkel I., Boder E., Braedt G., Charmley P., Concannon P., Ersoy F., Foroud T., Jaspers N. G., Lange K. Localization of an ataxia-telangiectasia gene to chromosome 11q22-23. Nature. 1988 Dec 8;336(6199):577–580. doi: 10.1038/336577a0. [DOI] [PubMed] [Google Scholar]
  9. Griffin C. A., McKeon C., Israel M. A., Gegonne A., Ghysdael J., Stehelin D., Douglass E. C., Green A. E., Emanuel B. S. Comparison of constitutional and tumor-associated 11;22 translocations: nonidentical breakpoints on chromosomes 11 and 22. Proc Natl Acad Sci U S A. 1986 Aug;83(16):6122–6126. doi: 10.1073/pnas.83.16.6122. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Jaspers N. G., Gatti R. A., Baan C., Linssen P. C., Bootsma D. Genetic complementation analysis of ataxia telangiectasia and Nijmegen breakage syndrome: a survey of 50 patients. Cytogenet Cell Genet. 1988;49(4):259–263. doi: 10.1159/000132673. [DOI] [PubMed] [Google Scholar]
  11. Kennedy M. A., Morris C. M., Fitzgerald P. H. The oncogene ETS1 is distant from the chromosome 11 breakpoint in leukaemic cells with the t(11;19)(q23;p13). Leuk Res. 1989;13(6):459–463. doi: 10.1016/0145-2126(89)90167-7. [DOI] [PubMed] [Google Scholar]
  12. Kerckaert J. P., Dozier C., Loucheux-Lefebvre M. H., Stehelin D. Sst I RFLP linked to the human ets-1 gene. Nucleic Acids Res. 1987 Jul 24;15(14):5905–5905. doi: 10.1093/nar/15.14.5905. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Lange K., Weeks D., Boehnke M. Programs for Pedigree Analysis: MENDEL, FISHER, and dGENE. Genet Epidemiol. 1988;5(6):471–472. doi: 10.1002/gepi.1370050611. [DOI] [PubMed] [Google Scholar]
  14. Morris C. M., Whitham S. E., Fitzgerald P. H. Transposition of the oncogene c-ets-1 in a t(11;19)(q23;p13) cell line transient during clonal evolution of blast crisis chronic myeloid leukemia. Leukemia. 1988 Feb;2(2):74–78. [PubMed] [Google Scholar]
  15. Peterson R. D., Funkhouser J. D. Speculations on ataxia-telangiectasia: defective regulation of the immunoglobulin gene superfamily. Immunol Today. 1989 Sep;10(9):313–314. doi: 10.1016/0167-5699(89)90087-X. [DOI] [PubMed] [Google Scholar]
  16. Pippard E. C., Hall A. J., Barker D. J., Bridges B. A. Cancer in homozygotes and heterozygotes of ataxia-telangiectasia and xeroderma pigmentosum in Britain. Cancer Res. 1988 May 15;48(10):2929–2932. [PubMed] [Google Scholar]
  17. Sacchi N., Perroni L., Papas T. S. A polymorphic SstI site within the human ets-1 gene in the 11q23 region. Nucleic Acids Res. 1986 Dec 9;14(23):9545–9545. doi: 10.1093/nar/14.23.9545. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Sacchi N., Watson D. K., Guerts van Kessel A. H., Hagemeijer A., Kersey J., Drabkin H. D., Patterson D., Papas T. S. Hu-ets-1 and Hu-ets-2 genes are transposed in acute leukemias with (4;11) and (8;21) translocations. Science. 1986 Jan 24;231(4736):379–382. doi: 10.1126/science.3941901. [DOI] [PubMed] [Google Scholar]
  19. Savage P. D., Hanson C. A., Kersey J. H. Identification of a restriction fragment length polymorphism involving the oncogene ETS-1 on chromosome 11q23. Blood. 1987 Jul;70(1):327–329. [PubMed] [Google Scholar]
  20. Swift M., Reitnauer P. J., Morrell D., Chase C. L. Breast and other cancers in families with ataxia-telangiectasia. N Engl J Med. 1987 May 21;316(21):1289–1294. doi: 10.1056/NEJM198705213162101. [DOI] [PubMed] [Google Scholar]
  21. Watson D. K., McWilliams-Smith M. J., Nunn M. F., Duesberg P. H., O'Brien S. J., Papas T. S. The ets sequence from the transforming gene of avian erythroblastosis virus, E26, has unique domains on human chromosomes 11 and 21: both loci are transcriptionally active. Proc Natl Acad Sci U S A. 1985 Nov;82(21):7294–7298. doi: 10.1073/pnas.82.21.7294. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Young B. R., Painter R. B. Radioresistant DNA synthesis and human genetic diseases. Hum Genet. 1989 May;82(2):113–117. doi: 10.1007/BF00284040. [DOI] [PubMed] [Google Scholar]
  23. Yunis J. J., Jones C., Madden M. T., Lu D., Mayer M. G. Gene order, amplification, and rearrangement of chromosome band 11q23 in hematologic malignancies. Genomics. 1989 Jul;5(1):84–90. doi: 10.1016/0888-7543(89)90090-6. [DOI] [PubMed] [Google Scholar]
  24. de Taisne C., Gegonne A., Stehelin D., Bernheim A., Berger R. Chromosomal localization of the human proto-oncogene c-ets. Nature. 1984 Aug 16;310(5978):581–583. doi: 10.1038/310581a0. [DOI] [PubMed] [Google Scholar]

Articles from American Journal of Human Genetics are provided here courtesy of American Society of Human Genetics

RESOURCES