| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 10823452 | DNA Repair | 2012 | 14 Pages |
Abstract
⺠Cockayne syndrome is a devasting childhood progeria most often caused by defects in the CSB gene. ⺠The CSB gene encodes a repair and chromatin remodeling SWI/SNF ATPase. ⺠The CSB gene also generates an abundant CSB-PGBD3 fusion protein that joins the N-terminus of CSB to a piggyBac transposase. ⺠The fusion protein affects DNA repair, and induces an interferon-like response in CSB-null cells. ⺠We speculate regarding the function of the conserved fusion protein in health and disease.
Keywords
Related Topics
Life Sciences
Biochemistry, Genetics and Molecular Biology
Biochemistry
Authors
Arnold D. Bailey, Lucas T. Gray, Thomas Pavelitz, John C. Newman, Katsuyoshi Horibata, Kiyoji Tanaka, Alan M. Weiner,