Article
Syndromes associated with mitochondrial DNA depletion.
Italian journal of pediatrics - 3 Apr 2014
Nogueira Célia, Almeida Ligia S, Nesti Claudia, Pezzini Ilaria, Videira Arnaldo, Vilarinho Laura, Santorelli Filippo M
Abstract excerpt
Mitochondrial dysfunction accounts for a large group of inherited metabolic disorders most of which are due to a dysfunctional mitochondrial respiratory chain (MRC) and, consequently, deficient energy production. MRC function depends on the coordinated expression of both nuclear (nDNA) and mitochondrial (mtDNA) genomes. Thus, mitochondrial diseases can be caused by genetic defects in either the mitochondrial or...
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