Skip to main content
PMC home page
American Journal of Human Genetics logoLink to American Journal of Human Genetics
. 1989 Apr;44(4):486–495.

A deletion map of the WAGR region on chromosome 11.

M Gessler 1, G H Thomas 1, P Couillin 1, C Junien 1, B C McGillivray 1, M Hayden 1, G Jaschek 1, G A Bruns 1
PMCID: PMC1715590  PMID: 2539014

Abstract

The WAGR (Wilms tumor, aniridia, genitourinary anomalies, and mental retardation) region has been assigned to chromosome 11p13 on the basis of overlapping constitutional deletions found in affected individuals. We have utilized 31 DNA probes which map to the WAGR deletion region, together with six reference loci and 13 WAGR-related deletions, to subdivide this area into 16 intervals. Specific intervals have been correlated with phenotypic features, leading to the identification of individual subregions for the aniridia and Wilms tumor loci. Delineation, by specific probes, of multiple intervals above and below the critical region and of five intervals within the overlap area provides a framework map for molecular characterization of WAGR gene loci and of deletion boundary regions.

Full text

PDF
486

Images in this article

489Figure 1
on p.489
490Figure 3
on p.490
491Figure 4
on p.491
492Figure 5
on p.492

Selected References

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

  1. Bickmore W., Christie S., van Heyningen V., Hastie N. D., Porteous D. J. Hitch-hiking from HRAS1 to the WAGR locus with CMGT markers. Nucleic Acids Res. 1988 Jan 11;16(1):51–60. doi: 10.1093/nar/16.1.51. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Brown W. R., Bird A. P. Long-range restriction site mapping of mammalian genomic DNA. 1986 Jul 31-Aug 6Nature. 322(6078):477–481. doi: 10.1038/322477a0. [DOI] [PubMed] [Google Scholar]
  3. Brunk C. F., Jones K. C., James T. W. Assay for nanogram quantities of DNA in cellular homogenates. Anal Biochem. 1979 Jan 15;92(2):497–500. doi: 10.1016/0003-2697(79)90690-0. [DOI] [PubMed] [Google Scholar]
  4. Bruns G. A., Mintz B. J., Leary A. C., Regina V. M., Gerald P. S. Human lysosomal genes: arylsulfatase A and beta-galactosidase. Biochem Genet. 1979 Dec;17(11-12):1031–1059. doi: 10.1007/BF00504344. [DOI] [PubMed] [Google Scholar]
  5. Church G. M., Gilbert W. Genomic sequencing. Proc Natl Acad Sci U S A. 1984 Apr;81(7):1991–1995. doi: 10.1073/pnas.81.7.1991. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Davis A. E., 3rd, Whitehead A. S., Harrison R. A., Dauphinais A., Bruns G. A., Cicardi M., Rosen F. S. Human inhibitor of the first component of complement, C1: characterization of cDNA clones and localization of the gene to chromosome 11. Proc Natl Acad Sci U S A. 1986 May;83(10):3161–3165. doi: 10.1073/pnas.83.10.3161. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Davis L. M., Stallard R., Thomas G. H., Couillin P., Junien C., Nowak N. J., Shows T. B. Two anonymous DNA segments distinguish the Wilms' tumor and aniridia loci. Science. 1988 Aug 12;241(4867):840–842. doi: 10.1126/science.2841760. [DOI] [PubMed] [Google Scholar]
  8. Douglass E. C., Wilimas J. A., Green A. A., Look A. T. Abnormalities of chromosomes 1 and 11 in Wilms' tumor. Cancer Genet Cytogenet. 1985 Jan 15;14(3-4):331–338. doi: 10.1016/0165-4608(85)90199-2. [DOI] [PubMed] [Google Scholar]
  9. Feder J., Yen L., Wijsman E., Wang L., Wilkins L., Schroder J., Spurr N., Cann H., Blumenberg M., Cavalli-Sforza L. L. A systematic approach for detecting high-frequency restriction fragment length polymorphisms using large genomic probes. Am J Hum Genet. 1985 Jul;37(4):635–649. [PMC free article] [PubMed] [Google Scholar]
  10. 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]
  11. Ferrell R. E., Riccardi V. M. Catalase levels in patients with aniridia and/or Wilms' tumor: utility and limitations. Cytogenet Cell Genet. 1981;31(2):120–123. doi: 10.1159/000131636. [DOI] [PubMed] [Google Scholar]
  12. Francke U., Holmes L. B., Atkins L., Riccardi V. M. Aniridia-Wilms' tumor association: evidence for specific deletion of 11p13. Cytogenet Cell Genet. 1979;24(3):185–192. doi: 10.1159/000131375. [DOI] [PubMed] [Google Scholar]
  13. Frischauf A. M., Murray N., Lehrach H. Lambda phage vectors--EMBL series. Methods Enzymol. 1987;153:103–115. doi: 10.1016/0076-6879(87)53051-8. [DOI] [PubMed] [Google Scholar]
  14. Gessler M., Bruns G. A. Molecular mapping and cloning of the breakpoints of a chromosome 11p14.1-p13 deletion associated with the AGR syndrome. Genomics. 1988 Aug;3(2):117–123. doi: 10.1016/0888-7543(88)90141-3. [DOI] [PubMed] [Google Scholar]
  15. Glaser T., Lewis W. H., Bruns G. A., Watkins P. C., Rogler C. E., Shows T. B., Powers V. E., Willard H. F., Goguen J. M., Simola K. O. The beta-subunit of follicle-stimulating hormone is deleted in patients with aniridia and Wilms' tumour, allowing a further definition of the WAGR locus. 1986 Jun 26-Jul 2Nature. 321(6073):882–887. doi: 10.1038/321882a0. [DOI] [PubMed] [Google Scholar]
  16. Gusella J. F., Keys C., VarsanyiBreiner A., Kao F. T., Jones C., Puck T. T., Housman D. Isolation and localization of DNA segments from specific human chromosomes. Proc Natl Acad Sci U S A. 1980 May;77(5):2829–2833. doi: 10.1073/pnas.77.5.2829. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Helms C., Graham M. Y., Dutchik J. E., Olson M. V. A new method for purifying lambda DNA from phage lysates. DNA. 1985 Feb;4(1):39–49. doi: 10.1089/dna.1985.4.39. [DOI] [PubMed] [Google Scholar]
  18. Junien C., Turleau C., Lenoir G. M., Philip T., Said R., Despoisse S., Laurent C., Rethoré M. O., Kaplan J. C., de Grouchy J. Catalase determination in various etiologic forms of Wilms' tumor and gonadoblastoma. Cancer Genet Cytogenet. 1983 Sep;10(1):51–57. doi: 10.1016/0165-4608(83)90105-x. [DOI] [PubMed] [Google Scholar]
  19. Junien C., Turleau C., de Grouchy J., Saïd R., Rethoré MO L., Tenconi R., Dufier J. L. Regional assignment of catalase (CAT) gene to band 11p13. Association with the aniridia-Wilms' tumor-Gonadoblastoma (WAGR) complex. Ann Genet. 1980;23(3):165–168. [PubMed] [Google Scholar]
  20. Kaneko Y., Egues M. C., Rowley J. D. Interstitial deletion of short arm of chromosome 11 limited to Wilms' tumor cells in a patient without aniridia. Cancer Res. 1981 Nov;41(11 Pt 1):4577–4578. [PubMed] [Google Scholar]
  21. Kunkel L. M., Tantravahi U., Eisenhard M., Latt S. A. Regional localization on the human X of DNA segments cloned from flow sorted chromosomes. Nucleic Acids Res. 1982 Mar 11;10(5):1557–1578. doi: 10.1093/nar/10.5.1557. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Moore J. W., Hyman S., Antonarakis S. E., Mules E. H., Thomas G. H. Familial isolated aniridia associated with a translocation involving chromosomes 11 and 22 [t(11;22)(p13;q12.2)]. Hum Genet. 1986 Apr;72(4):297–302. doi: 10.1007/BF00290952. [DOI] [PubMed] [Google Scholar]
  24. Porteous D. J., Bickmore W., Christie S., Boyd P. A., Cranston G., Fletcher J. M., Gosden J. R., Rout D., Seawright A., Simola K. O. HRAS1-selected chromosome transfer generates markers that colocalize aniridia- and genitourinary dysplasia-associated translocation breakpoints and the Wilms tumor gene within band 11p13. Proc Natl Acad Sci U S A. 1987 Aug;84(15):5355–5359. doi: 10.1073/pnas.84.15.5355. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Rogler C. E., Sherman M., Su C. Y., Shafritz D. A., Summers J., Shows T. B., Henderson A., Kew M. Deletion in chromosome 11p associated with a hepatitis B integration site in hepatocellular carcinoma. Science. 1985 Oct 18;230(4723):319–322. doi: 10.1126/science.2996131. [DOI] [PubMed] [Google Scholar]
  26. Sealey P. G., Whittaker P. A., Southern E. M. Removal of repeated sequences from hybridisation probes. Nucleic Acids Res. 1985 Mar 25;13(6):1905–1922. doi: 10.1093/nar/13.6.1905. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Shannon R. S., Mann J. R., Harper E., Harnden D. G., Morten J. E., Herbert A. Wilms's tumour and aniridia: clinical and cytogenetic features. Arch Dis Child. 1982 Sep;57(9):685–690. doi: 10.1136/adc.57.9.685. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Simola K. O., Knuutila S., Kaitila I., Pirkola A., Pohja P. Familial aniridia and translocation t(4;11)(q22;p13) without Wilms' tumor. Hum Genet. 1983;63(2):158–161. doi: 10.1007/BF00291536. [DOI] [PubMed] [Google Scholar]
  29. Turleau C., de Grouchy J., Nihoul-Fékété C., Dufier J. L., Chavin-Colin F., Junien C. Del11p13/nephroblastoma without aniridia. Hum Genet. 1984;67(4):455–456. doi: 10.1007/BF00291410. [DOI] [PubMed] [Google Scholar]
  30. Watkins P. C., Eddy R., Beck A. K., Vellucci V., Leverone B., Tanzi R. E., Gusella J. F., Shows T. B. DNA sequence and regional assignment of the human follicle-stimulating hormone beta-subunit gene to the short arm of human chromosome 11. DNA. 1987 Jun;6(3):205–212. doi: 10.1089/dna.1987.6.205. [DOI] [PubMed] [Google Scholar]
  31. de Martinville B., Francke U. The c-Ha-ras1, insulin and beta-globin loci map outside the deletion associated with aniridia-Wilms' tumour. Nature. 1983 Oct 13;305(5935):641–643. doi: 10.1038/305641a0. [DOI] [PubMed] [Google Scholar]

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

RESOURCES