Functional complementation of mitochondrial DNAs: Mobilizing mitochondrial genetics against dysfunction

• Published in: Biochimica et biophysica acta Vol. 1800; no. 3; pp. 245 - 249
• Main Authors: Schon, Eric A., Gilkerson, Robert W.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.03.2010
• Subjects: Adenosine Triphosphate - metabolism; Complementation; DNA, Circular - genetics; DNA, Mitochondrial - genetics; Genetic Complementation Test; Genetic Variation; Genome; Humans; Image Processing, Computer-Assisted; MELAS Syndrome - genetics; Mitochondria; Mitochondria - genetics; Mitochondria - metabolism; Mitochondria - pathology; Mitochondrial DNA; Mitochondrial Membranes - metabolism; Mitochondrial Membranes - pathology; Nucleoid; Oxygen Consumption; Transcription Factors - genetics; Transcription Factors - metabolism; Mitochondria; Mitochondrial DNA; Complementation; Nucleoid

Human mitochondrial DNA (mtDNA) is a 16.6-kb circular genome that is typically found in ∼ 1000 copies per cell. Frequently, one or more forms of mtDNA (i.e. wildtype (WT) and one or more mutant variants) will co-exist within an individual cell, a situation termed heteroplasmy; however, it has been u...