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Mitochondrial DNA transcription regulation and nucleoid organization

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Journal of Inherited Metabolic Disease

Abstract

Mitochondrial biogenesis is a complex process depending on both nuclear and mitochondrial DNA (mtDNA) transcription regulation to tightly coordinate mitochondrial levels and the cell’s energy demand. The energy requirements for a cell to support its metabolic function can change in response to varying physiological conditions, such as, proliferation and differentiation. Therefore, mitochondrial transcription regulation is constantly being modulated in order to establish efficient mitochondrial oxidative metabolism and proper cellular function. The aim of this article is to review the function of major protein factors that are directly involved in the process of mtDNA transcription regulation, as well as, the importance of mitochondrial nucleoid structure and its influence on mtDNA segregation and transcription regulation. Here, we discuss the current knowledge on the molecular mode of action of transcription factors comprising the mitochondrial transcriptional machinery, as well as the action of nuclear receptors on regulatory regions of the mtDNA.

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Acknowledgements

Our work was supported by PHS grant EY10804, NS041777, CA85700, The Parkinson Disease Foundation, the Florida James & Esther King Biomedical Research Program and the Muscular Dystrophy Association.

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  1. Departments of Neurology, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL, 33136, USA

    Adriana P. Rebelo & Carlos T. Moraes

  2. Departments of Cell Biology & Anatomy, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL, 33136, USA

    Adriana P. Rebelo, Lloye M. Dillon & Carlos T. Moraes

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  1. Adriana P. Rebelo
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Correspondence to Carlos T. Moraes.

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Communicated by: John Christodoulou

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Rebelo, A.P., Dillon, L.M. & Moraes, C.T. Mitochondrial DNA transcription regulation and nucleoid organization. J Inherit Metab Dis 34, 941–951 (2011). https://doi.org/10.1007/s10545-011-9330-8

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