LRPPRC and congenital lactic acidosis, Saguenay-Lac-Saint-Jean type: A homozygous c.1061C>T, p.(Ala354Val) founder mutation in the LRPPRC gene underpins the founder French-Canadian variant of Leigh Syndrome (LSFC) identified in the Saguenay-Lac-Saint-Jean region of Québec (Mootha et al., 2003).