[Free radical, lipid peroxide and antioxidant in mitochondrial encephalomyopathy].
We studied free radical, lipid peroxide (LPO) and antioxidant levels of blood in three cases with mitochondrial encephalomyopathy. Case 1 was a 17-year-old man with MELAS. Serum vitamin E levels were decreased and LPO levels were increased after stroke-like episodes in case 1. Case 2 was a 68-year-old woman with MELAS and a maternal elder aunt of case 1. She showed an elevated serum LPO levels (6.58 nmol/ml) in the absence of stroke-like episode and serum CoQ10 level was 0.54 microgram/ml before therapy. By CoQ10, idebenone and tocopherol nicotinate therapy, serum LPO levels decreased gradually in parallel with the decrease of lactate and pyruvate levels. Free radicals were measured in case 2 and controls by spin trapping method. Hydroxyl radical and C center radical were increased and H radical was normal in blood. But these free radicals in serum were all normal. Her serum antioxidants revealed an elevated percent inhibition of SOD and a decreased transfferin level. Case 3 was a 52-year-old woman with MERRF. She showed an elevation of serum LPO (12.8 nmol/ml). Her serum antioxidants revealed an elevated vitamin E and ceruloplasmin levels and percent inhibition of SOD.
Ihara
Y
Y
Clinical Research Institute, National Minamiokayama Hospital.
Hayabara
T
T
Namba
R
R
Nobukuni
K
K
Mori
A
A
D000293:Adolescent; D000368:Aged; D003067:Coenzymes; D005260:Female; D005609:Free Radicals / Q000097:blood; D006801:Humans; D008054:Lipid Peroxides / Q000097:blood*; D017241:MELAS Syndrome / Q000378:metabolism*; D017243:MERRF Syndrome / Q000378:metabolism*; D008297:Male; D013482:Superoxide Dismutase / Q000378:metabolism; D014451:Ubiquinone / Q000031:analogs & derivatives / Q000378:metabolism; D014810:Vitamin E / Q000097:blood
D003067:Coenzymes; D005609:Free Radicals; D008054:Lipid Peroxides; D014451:Ubiquinone; D014810:Vitamin E; D013482:Superoxide Dismutase; C024989:coenzyme Q10