Unknown

Dataset Information

0

Relationship of the xeroderma pigmentosum group E DNA repair defect to the chromatin and DNA binding proteins UV-DDB and replication protein A.


ABSTRACT: Cells from complementation groups A through G of the heritable sun-sensitive disorder xeroderma pigmentosum (XP) show defects in nucleotide excision repair of damaged DNA. Proteins representing groups A, B, C, D, F, and G are subunits of the core recognition and incision machinery of repair. XP group E (XP-E) is the mildest form of the disorder, and cells generally show about 50% of the normal repair level. We investigated two protein factors previously implicated in the XP-E defect, UV-damaged DNA binding protein (UV-DDB) and replication protein A (RPA). Three newly identified XP-E cell lines (XP23PV, XP25PV, and a line formerly classified as an XP variant) were defective in UV-DDB binding activity but had levels of RPA in the normal range. The XP-E cell extracts did not display a significant nucleotide excision repair defect in vitro, with either UV-irradiated DNA or a uniquely placed cisplatin lesion used as a substrate. Purified UV-DDB protein did not stimulate repair of naked DNA by DDB- XP-E cell extracts, but microinjection of the protein into DDB- XP-E cells could partially correct the repair defect. RPA stimulated repair in normal, XP-E, or complemented extracts from other XP groups, and so the effect of RPA was not specific for XP-E cell extracts. These data strengthen the connection between XP-E and UV-DDB. Coupled with previous results, the findings suggest that UV-DDB has a role in the repair of DNA in chromatin.

SUBMITTER: Rapic Otrin V 

PROVIDER: S-EPMC108900 | biostudies-literature | 1998 Jun

REPOSITORIES: biostudies-literature

altmetric image

Publications

Relationship of the xeroderma pigmentosum group E DNA repair defect to the chromatin and DNA binding proteins UV-DDB and replication protein A.

Rapić Otrin V V   Kuraoka I I   Nardo T T   McLenigan M M   Eker A P AP   Stefanini M M   Levine A S AS   Wood R D RD  

Molecular and cellular biology 19980601 6


Cells from complementation groups A through G of the heritable sun-sensitive disorder xeroderma pigmentosum (XP) show defects in nucleotide excision repair of damaged DNA. Proteins representing groups A, B, C, D, F, and G are subunits of the core recognition and incision machinery of repair. XP group E (XP-E) is the mildest form of the disorder, and cells generally show about 50% of the normal repair level. We investigated two protein factors previously implicated in the XP-E defect, UV-damaged  ...[more]

Similar Datasets

| S-EPMC2894533 | biostudies-literature
| S-EPMC2570873 | biostudies-literature
| S-EPMC2862104 | biostudies-literature
| S-EPMC3001049 | biostudies-literature
| S-EPMC2782015 | biostudies-literature
| S-EPMC6007576 | biostudies-literature
| S-EPMC3478663 | biostudies-literature
| S-EPMC10160032 | biostudies-literature
| S-EPMC3845163 | biostudies-literature
| S-EPMC3722669 | biostudies-literature