PMID:16278235

Citation	Thierbach, R, Schulz, TJ, Isken, F, Voigt, A, Mietzner, B, Drewes, G, von Kleist-Retzow, JC, Wiesner, RJ, Magnuson, MA, Puccio, H, Pfeiffer, AF, Steinberg, P and Ristow, M (2005) Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice. Hum. Mol. Genet. 14:3857-64
Abstract	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.
Links	PubMed Online version:10.1093/hmg/ddi410
Keywords	Animals; Apoptosis/genetics; Cell Proliferation; Hepatocytes/metabolism; Hepatocytes/pathology; Iron-Binding Proteins/genetics; Iron-Binding Proteins/metabolism; Iron-Sulfur Proteins/metabolism; Liver/pathology; Liver/physiology; Liver Neoplasms/genetics; Liver Neoplasms/pathology; Longevity/genetics; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria/genetics; Mitochondria/metabolism; Oxidative Stress; Phosphorylation; Reactive Oxygen Species/metabolism; p38 Mitogen-Activated Protein Kinases/metabolism
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