In the course of evolution, mitochondria lost their independence, and mtDNA became “slave” of nDNA, depending on numerous nucleus-encoded factors for its integrity, replication and expression. Mutations in any of these factors may alter the cross-talk between the two genomes and cause diseases that affect mtDNA integrity or expression, being inherited as mendelian traits.

Keywords:
Mitochondrial disease, Mitochondrial DNA, mtDNA depletion, mtDNA multiple deletions, Nucleus-mitochondrion cross-talk
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© 2007 The Biochemical Society
2007
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