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]. The gene discussed is LRPPRC; the disease is hyperinsulinemic hypoglycemia, familial, 4.