| First Author | Thierbach R | Year | 2005 |
| Journal | Hum Mol Genet | Volume | 14 |
| Issue | 24 | Pages | 3857-64 |
| PubMed ID | 16278235 | Mgi Jnum | J:104119 |
| Mgi Id | MGI:3611157 | Doi | 10.1093/hmg/ddi410 |
| Citation | Thierbach R, et al. (2005) Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice. Hum Mol Genet 14(24):3857-64 |
| abstractText | We have disrupted expression of the mitochondrial Friedreich ataxia protein frataxin specifically in murine hepatocytes to generate mice with impaired mitochondrial function and decreased oxidative phosphorylation. These animals have a reduced life span and develop multiple hepatic tumors. Livers also show increased oxidative stress, impaired respiration and reduced ATP levels paralleled by reduced activity of iron-sulfur cluster (Fe/S) containing proteins (ISP), which all leads to increased hepatocyte turnover by promoting both apoptosis and proliferation. Accordingly, phosphorylation of the stress-inducible p38 MAP kinase was found to be specifically impaired following disruption of frataxin. Taken together, these findings indicate that frataxin may act as a mitochondrial tumor suppressor protein in mammals. |
