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Article

Evidence for a repair enzyme complex involving ERCC1 and complementing activities of ERCC4, ERCC11 and xeroderma pigmentosum group F.

The EMBO journal - 1 Sept 1993

van Vuuren A J, Appeldoorn E, Odijk H, Yasui A, Jaspers N G, Bootsma D, Hoeijmakers J H

Abstract excerpt

Nucleotide excision repair (NER), one of the major cellular DNA repair systems, removes a wide range of lesions in a multi-enzyme reaction. In man, a NER defect due to a mutation in one of at least 11 distinct genes, can give rise to the inherited repair disorders xeroderma pigmentosum (XP), Cockayne's syndrome or PIBIDS, a photosensitive form of the brittle hair disease trichothiodystrophy. Laboratory-induced...

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Source

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Identifiers

PMID: 8253091
DOI: 10.1002/j.1460-2075.1993.tb06044.x
PMCID: PMC413646

Genes

ERCC1
ERCC4

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Evidence for a repair enzyme complex involving ERCC1 and complementing activities of ERCC4, ERCC11 and xeroderma pigmentosum group F.PMID 8253091Read ↗

Requirement for ERCC-1 and ERCC-3 gene products in DNA excision repair in vitro. Complementation using rodent and human cell extracts.Semantic proximity Read ↗

Activity of individual ERCC1 and XPF subunits in DNA nucleotide excision repair.Semantic proximity Read ↗

First reported patient with human ERCC1 deficiency has cerebro-oculo-facio-skeletal syndrome with a mild defect in nucleotide excision repair and severe developmental failure.Semantic proximity Read ↗

Do we know the cause of xeroderma pigmentosum?Semantic proximity Read ↗

UV-sensitive rodent mutant cell lines of complementation groups 6 and 8 differ phenotypically from their human counterparts.Semantic proximity Read ↗

Cells from ERCC1-deficient mice show increased genome instability and a reduced frequency of S-phase-dependent illegitimate chromosome exchange but a normal frequency of homologous recombination.Semantic proximity Read ↗

Phenotypic heterogeneity in nucleotide excision repair mutants of rodent complementation groups 1 and 4.Semantic proximity Read ↗

ERCC1 mutations impede DNA damage repair and cause liver and kidney dysfunction in patients.Semantic proximity Read ↗

Newly identified CHO ERCC3/XPB mutations and phenotype characterization.Semantic proximity Read ↗

Plant homologue of human excision repair gene ERCC1 points to conservation of DNA repair mechanisms.Semantic proximity Read ↗

Characterization of ERCC3 mutations in the Chinese hamster ovary 27-1, UV24 and MMC-2 cell lines.Semantic proximity Read ↗

The formation of UV-induced chromosome aberrations involves ERCC1 and XPF but not other nucleotide excision repair genes.Semantic proximity Read ↗

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