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

Journal Article · Tue Apr 26 04:00:00 EDT 1994 · Proceedings of the National Academy of Sciences of the United States of America

DOI:https://doi.org/10.1073/pnas.91.9.4053· OSTI ID:86512

Keeney, S; Brody, T; Linn, S ^[1]; Eker, A P.M.; Vermeulen, W; Bootsma, D; Hoeijmakers, J H.J. ^[2]

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1. Univ. of California, Berkeley, CA (United States)
2. Erasmus Univ., Rotterdam (Netherlands)

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.

• Sponsoring Organization: USDOE
• OSTI ID: 86512
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America, Journal Name: Proceedings of the National Academy of Sciences of the United States of America Journal Issue: 9 Vol. 91; ISSN PNASA6; ISSN 0027-8424
• Country of Publication: United States
• Language: English

Related Subjects

55 BIOLOGY AND MEDICINE
BASIC STUDIES
DNA REPAIR
GENETIC RADIATION EFFECTS
XERODERMA PIGMENTOSUM