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Proc Natl Acad Sci U S A. 1994 Apr 26;91(9):4053-6.

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

Author information

1
Division of Biochemistry and Molecular Biology, University of California, Berkeley 94720.

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.

PMID:
8171034
PMCID:
PMC43721
DOI:
10.1073/pnas.91.9.4053
[Indexed for MEDLINE]
Free PMC Article

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