Spinazzola Antonella, Zeviani Massimo
Unit of Molecular Neurogenetics, C. Besta Neurological Institute-Foundation IRCCS, via Libero Temolo 4, Milano, 20126, Italy.
Biosci Rep. 2007 Jun;27(1-3):39-51. doi: 10.1007/s10540-007-9036-1.
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.
在进化过程中，线粒体失去了独立性，线粒体DNA（mtDNA）成为核DNA（nDNA）的“奴隶”，其完整性、复制和表达依赖于众多由细胞核编码的因子。这些因子中任何一个发生突变，都可能改变两个基因组之间的相互作用，导致影响mtDNA完整性或表达的疾病，并作为孟德尔性状遗传。
