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Disorders of Nuclear-Mitochondrial Intergenomic Communication
Authors:Antonella Spinazzola  Massimo Zeviani
Affiliation:(1) Unit of Molecular Neurogenetics, “C. Besta” Neurological Institute—Foundation IRCCS, via Libero Temolo 4, Milano, 20126, Italy
Abstract: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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