The neglected genome

Mitochondria harbour some of the most critical functions of life [[1],[2],[3]]. As the site of ATP synthesis by oxidative phosphorylation, they are the primary energy‐generating system in almost all eukaryotic cells and are central to programmed cell death. Mitochondria also play major roles in a broad range of other key processes such as the synthesis of amino acids, haem, nucleotides and lipids, ion homeostasis, cell proliferation and motility. It is of major evolutionary and functional significance that they carry their own small DNA genome—a legacy of the endosymbiosis that created mitochondria, and possibly the eukaryotic cell, some 1.5–2 billion years ago [[4]]. In humans, mitochondrial DNA (mtDNA) is a double‐stranded circular molecule of 16.5 kilobase pairs that carries only 37 genes, 13 of …

[1]  D. Wallace,et al.  Mitochondrial DNA mutations in disease and aging , 2010, Environmental and molecular mutagenesis.

[2]  Nick Lane,et al.  The Costs of Breathing , 2011, Science.

[3]  V. Mootha,et al.  The mitochondrial proteome and human disease. , 2010, Annual review of genomics and human genetics.

[4]  John F. Allen,et al.  The function of genomes in bioenergetic organelles. , 2003, Philosophical transactions of the Royal Society of London. Series B, Biological sciences.

[5]  J. Chory,et al.  Coordination of gene expression between organellar and nuclear genomes , 2008, Nature Reviews Genetics.

[6]  L. Grivell.  Nucleo-mitochondrial interactions in mitochondrial gene expression. , 1995, Critical reviews in biochemistry and molecular biology.

[7]  P. Innocenti,et al.  Experimental Evidence Supports a Sex-Specific Selective Sieve in Mitochondrial Genome Evolution , 2011, Science.

[8]  A. Letai,et al.  Pretreatment Mitochondrial Priming Correlates with Clinical Response to Cytotoxic Chemotherapy , 2011, Science.

[9]  W. Martin,et al.  The energetics of genome complexity , 2010, Nature.

[10]  D. Jaffe,et al.  Molecular Diagnosis of Infantile Mitochondrial Disease with Targeted Next-Generation Sequencing , 2012, Science Translational Medicine.

[11]  D. Chan.  Mitochondria: Dynamic Organelles in Disease, Aging, and Development , 2006, Cell.

[12]  E. Culetto,et al.  Postfertilization Autophagy of Sperm Organelles Prevents Paternal Mitochondrial DNA Transmission , 2011, Science.

[13]  Miyuki Sato,et al.  Degradation of Paternal Mitochondria by Fertilization-Triggered Autophagy in C. elegans Embryos , 2011, Science.

[14]  D. Wallace,et al.  Energetics, epigenetics, mitochondrial genetics. , 2010, Mitochondrion.

[15]  T. Michael,et al.  METHODOLOGY ARTICLE Open Access , 2009 .