Disorders from perturbations of nuclear-mitochondrial intergenomic cross-talk.

TitleDisorders from perturbations of nuclear-mitochondrial intergenomic cross-talk.
Publication TypeJournal Article
Year of Publication2009
AuthorsSpinazzola, A, Zeviani, M
JournalJ Intern Med
Volume265
Issue2
Pagination174-92
Date Published2009 Feb
ISSN1365-2796
KeywordsAnimals, Disease Models, Animal, DNA, Mitochondrial, Gene Deletion, Humans, Mice, Mitochondrial Diseases, Mutation, Oxidative Phosphorylation
Abstract

In the course of evolution, mitochondria lost their independence, and mitochondrial DNA (mtDNA) became the 'slave' of nuclear DNA, 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 Mendelian disorders characterized by qualitative (multiple deletions) or quantitative (depletion) alterations of mtDNA, or by defective translation of mtDNA-encoded respiratory chain components.

DOI10.1111/j.1365-2796.2008.02059.x
Alternate JournalJ. Intern. Med.
Citation Key10.1111/j.1365-2796.2008.02059.x
PubMed ID19192035
Grant ListGGP07019 / / Telethon / Italy