1994
Correction of the DNA repair defect in xeroderma pigmentosum group E by injection of a DNA damage binding protein.
Publication
Publication
Proceedings of the National Academy of Sciences of the United States of America , Volume 91 p. 4053- 4056
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.
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hdl.handle.net/1765/3066 | |
Proceedings of the National Academy of Sciences of the United States of America | |
Organisation | Erasmus MC: University Medical Center Rotterdam |
Keeney, S., Eker, A., Brody, T., Vermeulen, W., Bootsma, D., Hoeijmakers, J., & Linn, S. (1994). Correction of the DNA repair defect in xeroderma pigmentosum group E by injection of a DNA damage binding protein. Proceedings of the National Academy of Sciences of the United States of America, 91, 4053–4056. Retrieved from http://hdl.handle.net/1765/3066 |