UVSSA

UV-stimulated scaffold protein A [KIAA1530]

Publications

A C. elegans homolog for the UV-hypersensitivity syndrome disease gene UVSSA.

PubMed

Full text

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