This mutation causes a specific defect of UUG-rich gene translation such as ND6 gene that encodes a subunit of the NADH-coenzyme Q reductase complex, also known as Complex I. This translation defect results in specific Complex I deficiency due to a reduction in the synthesis of ND6 subunit that is characteristic of the MELAS syndrome (Kirino and Suzuki, 2005). The gene discussed is MT-ND6; the disease is MELAS syndrome.