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Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice

  • 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

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Author:Renè Thierbach, Tim Julius SchulzORCiDGND, Frank Isken, Aanja Voigt, Brun Mietzner, Gunnar DrewesGND, Jürgen-Christoph von Kleist-Retzow, Rudolf J. Wiesner, Mark A. Magnuson, Helene Puccio, Andreas F.H. Pfeiffer, Pablo Steinberg, Michael Ristow
Document Type:Article
Language:English
Year of first Publication:2005
Year of Completion:2005
Release Date:2017/03/24
Source:Human molecular genetics. - 14 (2005), 24, S. 3857 - 3864
Organizational units:Mathematisch-Naturwissenschaftliche Fakultät / Institut für Ernährungswissenschaft
Peer Review:Referiert