Abstract
The transcription-coupled repair pathway (TC-NER) plays a vital role in removing transcription-blocking DNA lesions, particularly UV-induced damage. Clinical symptoms of the two TC-NER-deficiency syndromes, Cockayne syndrome (CS) and UV-hypersensitivity syndrome (UVSS) are dissimilar and the underlying molecular mechanism causing this difference in disease pathology is not yet clearly understood. UV-stimulated scaffold protein A (UVSSA) has been identified recently as a new causal gene for UVSS. Here we describe a functional homolog of the human UVSSA gene in the nematode Caenorhabditis elegans, uvs-1 (UVSSA-like-1). Mutations in uvs-1 render the animals hypersensitive to UV-B irradiation and transcription-blocking lesion-inducing illudin-M, similar to mutations in TC-NER deficient mutants. Moreover, we demonstrate that TC-NER factors including UVS-1 are required for the survival of the adult animals after UV-treatment.
Original language | English |
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Pages (from-to) | 8-15 |
Number of pages | 8 |
Journal | DNA Repair |
Volume | 41 |
Early online date | 25 Mar 2016 |
DOIs | |
Publication status | Published - 01 May 2016 |
Externally published | Yes |
Keywords
- C. elegans
- Cockayne syndrome
- DNA damage
- Nucleotide excision repair
- Ultraviolet light
- UV-hypersensitivity syndrome
ASJC Scopus subject areas
- Biochemistry
- Molecular Biology
- Cell Biology