Transcriptional Control of Striated Muscle Mitochondrial Biogenesis and Function

Heart and skeletal muscle must satisfy tremendous energy demands following birth in order to provide persistent pump and locomotor functions. The high energy requirements of striated muscle are met through a specialized, high-capacity mitochondrial system. Significant progress has been made in defining the gene regulatory network controlling mitochondrial biogenesis and function in striated muscle. Transcription factors encoded in the nucleus direct the activity of genes located in both the nuclear and mitochondrial genomes. This transcriptional regulatory circuitry responds to developmental and physiological cues. Emerging evidence suggests that dysregulation of this regulatory network contributes to common diseases of the heart and skeletal muscle. This chapter reviews and summarizes current knowledge about this important gene regulatory circuitry relevant to health and diseases of the heart and skeletal muscle.