The leucine-rich pentatricopeptide repeat containing (LRPPRC) protein regulates mitochondrial mRNA stability and an amino-acid substitution of this protein causes the French-Canadian type of Leigh syndrome (LSFC), a progressive neurodegenerative disorder characterized by mitochondrial complex IV deficiency [27, 28]. This evidence concerns the gene LRPPRC and congenital lactic acidosis, Saguenay-Lac-Saint-Jean type.