COQ6 and Hypoalbuminemia: In the present study, we report two novel pathogenic COQ6 mutations: c.249C > G (p.Tyr83Ter) and c.1381C > T (p.Gln461Ter), which, in a compound heterozygous state, resulted in two siblings with clinical presentations of COQ10D6 characterized by renal involvements including hypoalbuminemia, proteinuria and edema, and extra-renal manifestations including growth retardation, muscle hypotonia, and/or seizures.