Third, in other mitochondrial diseases, such as myoclonus epilepsy associated with ragged-red fibers (MERRF), mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS), mitochondrion are fragmented which is associated with proteolytical processing of OPA1[17]. The gene discussed is OPA1; the disease is inborn mitochondrial metabolism disorder.