ABSTRACT

Abstract ....................................................................................................................68 A Modern View of the Mitochondrion .....................................................................68 Mitochondrial DNA Replication and Transcription ................................................. 71

Overview of the Molecular Machinery ............................................................... 71 Mitochondrial Transcription Factor A ................................................................. 72 Mitochondrial Transcription Factor B2: TFB1M and TFB2M ........................... 72 Mitochondrial RNA Polymerase ......................................................................... 72 Mitochondrial Transcription Termination Factors Family .................................. 73 Mitochondrial Transcription Elongation Factor .................................................. 73 Nuclear-Encoded Factors Acting within Mitochondria ...................................... 73

Transcriptional Regulation of Mitochondrial Biogenesis ........................................ 74 Nuclear Respiratory Activators ........................................................................... 74 Mitochondrial Transcriptional Control by Myc .................................................. 75 Glucocorticoids, Thyroid Hormone, and Other Steroid Hormones .................... 76 Nuclear Hormone Receptor Activators of Mitochondrial Biogenesis.................77 Other Nuclear Transcription Factors ................................................................... 78 PGC-1 Coactivator Family .................................................................................. 79

Transcriptional Repression of Mitochondrial Biogenesis ........................................ 82 Mitophagy ................................................................................................................ 83 Coregulation of Mitophagy and Mitochondrial Biogenesis .....................................85 Mitochondrial Biogenesis in Disease .......................................................................85