Abdelhakim et al. [40] described three siblings from a Jewish family presenting with nephropathy and retinopathy, resulting from two COQ2 mutations ([c.288dupC] [p.(Ala97Argfs*56] and [c.376C>G] [p.(Arg126Gly]); supplementation with CoQ10 (30 mg/kg/day for 6 months) prevented further visual deterioration in these patients. Here, COQ2 is linked to Nephropathy.