A C. elegans homolog for the UV-hypersensitivity syndrome disease gene UVSSA |
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| Affiliation: | 1. Institute for Genome Stability in Ageing and Disease, Medical Faculty, University of Cologne, 50931 Cologne, Germany;2. Cologne Excellence Cluster for Cellular Stress Responses in Ageing-Associated Diseases (CECAD), Research Center and Centre for Molecular Medicine (CMMC), University of Cologne, Joseph-Stelzmann-Str. 26, 50931 Cologne, Germany;1. School of Applied Sciences, University of Campinas, Limeira, SP, Brazil;2. Post-Graduate Program in Movement Sciences, São Paulo State University (Unesp), Institute of Biosciences, Rio Claro, Sao Paulo, Brazil;3. School of Physical Education and Sport of Ribeirao Preto, University of Sao Paulo, Ribeirao Preto, Sao Paulo, Brazil;1. Max Planck Institute for Biophysical Chemistry, Am Fassberg 11, 37077 Göttingen, Germany |
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| 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. |
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| Keywords: | Nucleotide excision repair Cockayne syndrome UV-hypersensitivity syndrome Ultraviolet light DNA damage |
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