UVSSA
UV-stimulated scaffold protein A [KIAA1530]
Publications[править]
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.
MeSH Terms
- Animals
- Caenorhabditis elegans
- Caenorhabditis elegans Proteins
- Carrier Proteins
- DNA Repair
- Humans
- Longevity
- Mutation
- Oxidative Stress
- Polycyclic Sesquiterpenes
- Sequence Homology, Nucleic Acid
- Sesquiterpenes
- Transcription, Genetic
- Ultraviolet Rays
Keywords
- C. elegans
- Cockayne syndrome
- DNA damage
- Nucleotide excision repair
- UV-hypersensitivity syndrome
- Ultraviolet light