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KMID : 0381120100320050455
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Genes and Genomics
2010 Volume.32 No. 5 p.455 ~ p.461
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Mitochondrial transcription factor B1 is required for mitochondrial function and oxidative stress resistance in Drosophila
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Kim Hyun-Jin
Kim Min-Ju
Jeong Ji-Eun
Chung Jong-Kyeong
Lee Hye-Jeong
Koh Hyong-Jong
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Abstract
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Mitochondrial protein synthesis is critical in biogenesis of electron transport chain and maintenance of mitochondrial function. Mitochondrial transcription factor B1 (TFB1M), an in vitro mitochondrial transcription activator, has been identified as an important mitochondrial translation regulator in vivo. To elucidate its physiological function, we generated and characterized Drosophila TFB1M mutants. Although TFB1M mutants showed normal development, decreased ATP level and reduced activity of complex IV, the last enzyme in electron transport chain, were observed in the indirect flight muscle of TFB1M mutants. Moreover, TFB1M mutants showed an increased sensitivity to oxidative stress and a significant accumulation of oxidative damage. In addition to these TFB1M mutant phenotypes, over-expression of TFB1M substantially ameliorated mitochondria dysfunction and the disease-related phenotypes in Drosophila Parkinson’s disease (PD) models. These data demonstrate the role of TFB1M in maintaining mitochondrial function and oxidative stress resistance.
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KEYWORD
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TFB1M, Mitochondrial biogenesis, Oxidative stress Electron transport chain, Mitochondrial dysfunction
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