Starr et al. [38] reported three children (aged 2–10 years) with nephrotic syndrome resulting from COQ2 mutations (two missense (p.Thr325Ala and p.Thr294Ile), and one frameshift (c.176dupT), in two of whom renal function was restored following CoQ10 supplementation (30 mg/kg/day). This evidence concerns the gene COQ2 and nephrotic syndrome.