Oxidative damage to muscle protein in Duchenne muscular dystrophy.
The hypothesis that reactive free radical species (ROS) may contribute to the pathogenesis of Duchenne muscular dystrophy (DMD) has been suggested previously, but experimental data obtained in support of the above have to date proved inconclusive. The recent discovery that nitric oxide synthase (required for muscle relaxation) is associated with the sarcolemmal protein dystrophin normally and that both proteins are absent in DMD has heightened interest in the potential role of ROS in this disorder. We therefore investigated oxidative damage to proteins in the quadriceps femoris muscle by quantifying protein carbonyl levels in six patients with DMD and six normal controls. In DMD, the mean protein carbonyl level in the quadriceps femoris muscle was increased by 211% (p < 0.005) compared with the normal control subjects. The data thus support the hypothesis for the role of ROS induced protein oxidation of muscle cell damage in DMD.
Haycock
J W
JW
University Department of Medicine, Clinical Sciences Centre, Northern General Hospital, Sheffield, UK.
MacNeil
S
S
Jones
P
P
Harris
J B
JB
Mantle
D
D
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D006801:Humans; D017665:Hydroxyl Radical / Q000378:metabolism; D066298:In Vitro Techniques; D007223:Infant; D008297:Male; D009124:Muscle Proteins / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000378:metabolism; D009136:Muscular Dystrophies / Q000378:metabolism*; D018384:Oxidative Stress / Q000502:physiology*; D010659:Phenylhydrazines / Q000494:pharmacology; D012508:Sarcolemma / Q000378:metabolism; D013481:Superoxides / Q000378:metabolism
D009124:Muscle Proteins; D010659:Phenylhydrazines; D013481:Superoxides; C004787:2,4-dinitrophenylhydrazine; D017665:Hydroxyl Radical