Disorders of nuclear-mitochondrial intergenomic communication.
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.
Spinazzola
Antonella
A
Unit of Molecular Neurogenetics, C. Besta Neurological Institute-Foundation IRCCS, via Libero Temolo 4, Milano, 20126, Italy.
Zeviani
Massimo
M
D002467:Cell Nucleus / Q000235:genetics*; D004272:DNA, Mitochondrial / Q000235:genetics*; D042822:Genomic Instability; D006801:Humans; D028361:Mitochondrial Diseases / Q000235:genetics* / Q000473:pathology; D009154:Mutation; D014176:Protein Biosynthesis
D004272:DNA, Mitochondrial