期刊
CELL DEATH & DISEASE
卷 2, 期 -, 页码 -出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/cddis.2011.5
关键词
frataxin; hypoxia; p53; HIF; Friedreich's ataxia; tumor
类别
资金
- FIRB
- Atena Onlus Association
- Regione Lazio
- National Ataxia Foundation, Ataxia UK
- Friedreich's Ataxia Research Alliance
- Telethon-Italy [GGP06059]
- Association Francaise de l'Ataxie de Friedreich (AFAF)
Defective expression of frataxin is responsible for the degenerative disease Friedreich's ataxia. Frataxin is a protein required for cell survival since complete knockout is lethal. Frataxin protects tumor cells against oxidative stress and apoptosis but also acts as a tumor suppressor. The molecular bases of this apparent paradox are missing. We therefore sought to investigate the pathways through which frataxin enhances stress resistance in tumor cells. We found that frataxin expression is upregulated in several tumor cell lines in response to hypoxic stress, a condition often associated with tumor progression. Moreover, frataxin upregulation in response to hypoxia is dependent on hypoxia-inducible factors expression and modulates the activation of the tumor-suppressor p53. Importantly, we show for the first time that frataxin is in fact increased in human tumors in vivo. These results show that frataxin participates to the hypoxia-induced stress response in tumors, thus implying that modulation of its expression could have a critical role in tumor cell survival and/or progression. Cell Death and Disease (2011) 2, e123; doi: 10.1038/cddis.2011.5; published online 24 February 2011
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据