title: Repair of some active genes in Cockayne syndrome cells is at the genome overall rate.
abstract: Repair of UV (254 nm)-induced DNA damage in cells from patients with the genetic disease Cockayne syndrome (CS; CS3BE, CS2BE) has been examined in several different genomic regions. These regions include those that contain the actively transcribed beta-actin and adenosine deaminase (ADA) genes and the inactive insulin and 754 loci. The beta-actin, ADA and insulin regions are repaired at about the same rate, one which is equal to the genome overall repair rate. The 754 locus is repaired considerably more slowly. The insulin region is repaired at the same rate in both CS and normal cells as is the 754 locus. The only difference from normal is that the active genes, while repaired well, are not preferentially repaired relative to the genome overall. Our results are consistent with the hypothesis that the repair defect in CS is due to an inactive transcription-repair coupling factor (TRCF). However, the results also indicate that factors other than TRCF and active transcription must also promote repair of some regions relative to others in both normal and CS cells.
authors.affiliation: Department of Biological Sciences, Wright State University, Dayton, OH 45435, USA.
authors.forename: G J
authors.initials: GJ
authors.lastname: Kantor
authors.forename: S A
authors.initials: SA
authors.lastname: Bastin
mesh_terms: D000199:Actins / Q000235:genetics; D000243:Adenosine Deaminase / Q000235:genetics; D002478:Cells, Cultured; D003057:Cockayne Syndrome / Q000235:genetics*; D004249:DNA Damage; D004260:DNA Repair / Q000235:genetics* / Q000502:physiology; D005347:Fibroblasts / Q000528:radiation effects; D006801:Humans; D007328:Insulin / Q000235:genetics; D013762:Tetrahydrofolate Dehydrogenase / Q000235:genetics; D013997:Time Factors; D014466:Ultraviolet Rays; D014960:X Chromosome / Q000235:genetics
chemical_list: D000199:Actins; D007328:Insulin; D013762:Tetrahydrofolate Dehydrogenase; D000243:Adenosine Deaminase