| First Author | Shackelford RE | Year | 2004 |
| Journal | DNA Repair (Amst) | Volume | 3 |
| Issue | 10 | Pages | 1263-72 |
| PubMed ID | 15336622 | Mgi Jnum | J:92530 |
| Mgi Id | MGI:3053470 | Doi | 10.1016/j.dnarep.2004.01.015 |
| Citation | Shackelford RE, et al. (2004) Iron chelators increase the resistance of Ataxia telangeictasia cells to oxidative stress. DNA Repair (Amst) 3(10):1263-72 |
| abstractText | Ataxia telangeictasia (A-T) is an autosomal recessive disorder characterized by immune dysfunction, genomic instability, chronic oxidative damage, and increased cancer incidence. Previously, desferal was found to increase the resistance of A-T, but not normal cells to exogenous oxidative stress in the colony forming-efficiency assay, suggesting that iron metabolism is dysregulated in A-T. Since desferal both chelates iron and modulates gene expression, we tested the effects of apoferritin and the iron chelating flavonoid quercetin on A-T cell colony-forming ability. We demonstrate that apoferritin and quercetin increase the ability of A-T cells to form colonies. We also show that labile iron levels are significantly elevated in Atm-deficient mouse sera compared to syngeniec wild type mice. Our findings support a role for labile iron acting as a Fenton catalyst in A-T, contributing to the chronic oxidative stress seen in this disease. Our findings further suggest that iron chelators might promote the survival of A-T cells and hence, individuals with A-T. |
