UVSSA

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UV-stimulated scaffold protein A [KIAA1530]

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

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