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Disorders from perturbations of nuclear‐mitochondrial intergenomic cross‐talk
Author(s) -
Spinazzola A.,
Zeviani M.
Publication year - 2009
Publication title -
journal of internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.625
H-Index - 160
eISSN - 1365-2796
pISSN - 0954-6820
DOI - 10.1111/j.1365-2796.2008.02059.x
Subject(s) - mitochondrial dna , nuclear dna , genetics , mendelian inheritance , mitochondrion , biology , dna , cell nucleus , mitochondrial disease , genome , heteroplasmy , nuclear gene , non mendelian inheritance , gene
. 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.