8-hydroxy-2--deoxyguanosine and MELAS-Syndrome

To investigate the relationship between oxidative stress and progressive spread of the stroke-like lesions in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with 3243A>G mutation, we retrospectively analyzed the spread frequency in patients with and without treatment with the radical scavenger edaravone. Oxidative damage and defensive enzymes were histologically evaluated. Spread was significantly less frequent in the patients treated with edaravone. Although 8-hydroxy-2'-deoxyguanosine, a marker for oxidative damage of DNA, was obviously accumulated in peri-lesional surviving neurons, manganese superoxide dismutase and 8-oxoguanine glycosylase 1 were not up-regulated in those neurons. Increased oxidative stress and insufficient defense could be involved in the pathogenesis of the spreading lesions in MELAS.

Topics: 8-Hydroxy-2'-Deoxyguanosine; Adolescent; Adult; Aged; Aged, 80 and over; Antipyrine; Deoxyguanosine; DNA Glycosylases; Edaravone; Female; Free Radical Scavengers; Histocytochemistry; Humans; Male; MELAS Syndrome; Middle Aged; Neurons; Oxidative Stress; Superoxide Dismutase; Young Adult