Skip to main content
PMC home page
Proceedings of the National Academy of Sciences of the United States of America logoLink to Proceedings of the National Academy of Sciences of the United States of America
. 1994 Apr 26;91(9):4053–4056. doi: 10.1073/pnas.91.9.4053

Correction of the DNA repair defect in xeroderma pigmentosum group E by injection of a DNA damage-binding protein.

S Keeney 1, A P Eker 1, T Brody 1, W Vermeulen 1, D Bootsma 1, J H Hoeijmakers 1, S Linn 1
PMCID: PMC43721  PMID: 8171034

Abstract

Cells from a subset of patients with the DNA-repair-defective disease xeroderma pigmentosum complementation group E (XP-E) are known to lack a DNA damage-binding (DDB) activity. Purified human DDB protein was injected into XP-E cells to test whether the DNA-repair defect in these cells is caused by a defect in DDB activity. Injected DDB protein stimulated DNA repair to normal levels in those strains that lack the DDB activity but did not stimulate repair in cells from other xeroderma pigmentosum groups or in XP-E cells that contain the activity. These results provide direct evidence that defective DDB activity causes the repair defect in a subset of XP-E patients, which in turn establishes a role for this activity in nucleotide-excision repair in vivo.

Full text

PDF
4053

Images in this article

4054Image
on p.4054

Selected References

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

  1. Brown S. J., Kellett P. J., Lippard S. J. Ixr1, a yeast protein that binds to platinated DNA and confers sensitivity to cisplatin. Science. 1993 Jul 30;261(5121):603–605. doi: 10.1126/science.8342024. [DOI] [PubMed] [Google Scholar]
  2. Chao C. C., Huang S. L., Lee L. Y., Lin-Chao S. Identification of inducible damage-recognition proteins that are overexpressed in HeLa cells resistant to cis-diamminedichloroplatinum (II). Biochem J. 1991 Aug 1;277(Pt 3):875–878. doi: 10.1042/bj2770875. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Chao C. C., Lee Y. L., Cheng P. W., Lin-Chao S. Enhanced host cell reactivation of damaged plasmid DNA in HeLa cells resistant to cis-diamminedichloroplatinum(II). Cancer Res. 1991 Jan 15;51(2):601–605. [PubMed] [Google Scholar]
  4. Chu G., Chang E. Cisplatin-resistant cells express increased levels of a factor that recognizes damaged DNA. Proc Natl Acad Sci U S A. 1990 May;87(9):3324–3327. doi: 10.1073/pnas.87.9.3324. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Chu G., Chang E. Xeroderma pigmentosum group E cells lack a nuclear factor that binds to damaged DNA. Science. 1988 Oct 28;242(4878):564–567. doi: 10.1126/science.3175673. [DOI] [PubMed] [Google Scholar]
  6. Donahue B. A., Augot M., Bellon S. F., Treiber D. K., Toney J. H., Lippard S. J., Essigmann J. M. Characterization of a DNA damage-recognition protein from mammalian cells that binds specifically to intrastrand d(GpG) and d(ApG) DNA adducts of the anticancer drug cisplatin. Biochemistry. 1990 Jun 19;29(24):5872–5880. doi: 10.1021/bi00476a032. [DOI] [PubMed] [Google Scholar]
  7. Eker A. P., Vermeulen W., Miura N., Tanaka K., Jaspers N. G., Hoeijmakers J. H., Bootsma D. Xeroderma pigmentosum group A correcting protein from calf thymus. Mutat Res. 1992 Sep;274(3):211–224. doi: 10.1016/0921-8777(92)90067-d. [DOI] [PubMed] [Google Scholar]
  8. Feldberg R. S., Grossman L. A DNA binding protein from human placenta specific for ultraviolet damaged DNA. Biochemistry. 1976 Jun 1;15(11):2402–2408. doi: 10.1021/bi00656a024. [DOI] [PubMed] [Google Scholar]
  9. Hirschfeld S., Levine A. S., Ozato K., Protić M. A constitutive damage-specific DNA-binding protein is synthesized at higher levels in UV-irradiated primate cells. Mol Cell Biol. 1990 May;10(5):2041–2048. doi: 10.1128/mcb.10.5.2041. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Hoeijmakers J. H., Eker A. P., Wood R. D., Robins P. Use of in vivo and in vitro assays for the characterization of mammalian excision repair and isolation of repair proteins. Mutat Res. 1990 Sep-Nov;236(2-3):223–238. doi: 10.1016/0921-8777(90)90007-r. [DOI] [PubMed] [Google Scholar]
  11. Hughes E. N., Engelsberg B. N., Billings P. C. Purification of nuclear proteins that bind to cisplatin-damaged DNA. Identity with high mobility group proteins 1 and 2. J Biol Chem. 1992 Jul 5;267(19):13520–13527. [PubMed] [Google Scholar]
  12. Hwang B. J., Chu G. Purification and characterization of a human protein that binds to damaged DNA. Biochemistry. 1993 Feb 16;32(6):1657–1666. doi: 10.1021/bi00057a033. [DOI] [PubMed] [Google Scholar]
  13. Kataoka H., Fujiwara Y. UV damage-specific DNA-binding protein in xeroderma pigmentosum complementation group E. Biochem Biophys Res Commun. 1991 Mar 29;175(3):1139–1143. doi: 10.1016/0006-291x(91)91684-5. [DOI] [PubMed] [Google Scholar]
  14. Keeney S., Chang G. J., Linn S. Characterization of a human DNA damage binding protein implicated in xeroderma pigmentosum E. J Biol Chem. 1993 Oct 5;268(28):21293–21300. [PubMed] [Google Scholar]
  15. Keeney S., Wein H., Linn S. Biochemical heterogeneity in xeroderma pigmentosum complementation group E. Mutat Res. 1992 Jan;273(1):49–56. doi: 10.1016/0921-8777(92)90049-9. [DOI] [PubMed] [Google Scholar]
  16. Kondo S., Mamada A., Miyamoto C., Keong C. H., Satoh Y., Fujiwara Y. Late onset of skin cancers in 2 xeroderma pigmentosum group F siblings and a review of 30 Japanese xeroderma pigmentosum patients in groups D, E and F. Photodermatol. 1989 Apr;6(2):89–95. [PubMed] [Google Scholar]
  17. Pil P. M., Lippard S. J. Specific binding of chromosomal protein HMG1 to DNA damaged by the anticancer drug cisplatin. Science. 1992 Apr 10;256(5054):234–237. doi: 10.1126/science.1566071. [DOI] [PubMed] [Google Scholar]
  18. Reardon J. T., Nichols A. F., Keeney S., Smith C. A., Taylor J. S., Linn S., Sancar A. Comparative analysis of binding of human damaged DNA-binding protein (XPE) and Escherichia coli damage recognition protein (UvrA) to the major ultraviolet photoproducts: T[c,s]T, T[t,s]T, T[6-4]T, and T[Dewar]T. J Biol Chem. 1993 Oct 5;268(28):21301–21308. [PubMed] [Google Scholar]
  19. Sancar A., Franklin K. A., Sancar G. B. Escherichia coli DNA photolyase stimulates uvrABC excision nuclease in vitro. Proc Natl Acad Sci U S A. 1984 Dec;81(23):7397–7401. doi: 10.1073/pnas.81.23.7397. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Sancar G. B., Smith F. W. Interactions between yeast photolyase and nucleotide excision repair proteins in Saccharomyces cerevisiae and Escherichia coli. Mol Cell Biol. 1989 Nov;9(11):4767–4776. doi: 10.1128/mcb.9.11.4767. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Stefanini M., Giliani S., Nardo T., Marinoni S., Nazzaro V., Rizzo R., Trevisan G. DNA repair investigations in nine Italian patients affected by trichothiodystrophy. Mutat Res. 1992 Mar;273(2):119–125. doi: 10.1016/0921-8777(92)90073-c. [DOI] [PubMed] [Google Scholar]
  22. Stefanini M., Vermeulen W., Weeda G., Giliani S., Nardo T., Mezzina M., Sarasin A., Harper J. I., Arlett C. F., Hoeijmakers J. H. A new nucleotide-excision-repair gene associated with the disorder trichothiodystrophy. Am J Hum Genet. 1993 Oct;53(4):817–821. [PMC free article] [PubMed] [Google Scholar]
  23. Takao M., Abramic M., Moos M., Jr, Otrin V. R., Wootton J. C., McLenigan M., Levine A. S., Protic M. A 127 kDa component of a UV-damaged DNA-binding complex, which is defective in some xeroderma pigmentosum group E patients, is homologous to a slime mold protein. Nucleic Acids Res. 1993 Aug 25;21(17):4111–4118. doi: 10.1093/nar/21.17.4111. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Vermeulen W., Osseweijer P., de Jonge A. J., Hoeijmakers J. H. Transient correction of excision repair defects in fibroblasts of 9 xeroderma pigmentosum complementation groups by microinjection of crude human cell extracts. Mutat Res. 1986 May;165(3):199–206. doi: 10.1016/0167-8817(86)90055-6. [DOI] [PubMed] [Google Scholar]
  25. Vermeulen W., Stefanini M., Giliani S., Hoeijmakers J. H., Bootsma D. Xeroderma pigmentosum complementation group H falls into complementation group D. Mutat Res. 1991 Sep;255(2):201–208. doi: 10.1016/0921-8777(91)90054-s. [DOI] [PubMed] [Google Scholar]
  26. de Jonge A. J., Vermeulen W., Klein B., Hoeijmakers J. H. Microinjection of human cell extracts corrects xeroderma pigmentosum defect. EMBO J. 1983;2(5):637–641. doi: 10.1002/j.1460-2075.1983.tb01477.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. de Weerd-Kastelein E. A., Keijzer W., Bootsma D. A third complementation group in xeroderma pigmentosum. Mutat Res. 1974 Jan;22(1):87–91. doi: 10.1016/0027-5107(74)90013-x. [DOI] [PubMed] [Google Scholar]
  28. van Assendelft G. B., Rigney E. M., Hickson I. D. Purification of a HeLa cell nuclear protein that binds selectively to DNA irradiated with ultra-violet light. Nucleic Acids Res. 1993 Jul 25;21(15):3399–3404. doi: 10.1093/nar/21.15.3399. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Proceedings of the National Academy of Sciences of the United States of America are provided here courtesy of National Academy of Sciences

RESOURCES