SearchPublisher and Institutes
You are here: Home :: Area NEM :: Medical science :: Human medicine
Gabriele Siciliano, Livia Pasquali, Anna Rocchi, Michela Falorni, Fabio Galluzzi, Angela Rocco, Gino Malvaldi, Alfonso Pompella, Aldo Paolicchi
Advanced oxidation protein products in serum of patients with myotonic disease type I: association with serum ?-glutamyltransferase and disease severity
Keywords: ?-glutamyltransferase, myotonic dystrophy, oxidative stress
Steinert's disease (myotonic dystrophy type 1; MD) is caused by a CTG trinucleotide expansion on 19q13.3. Although the pathogenic mechanism underlying multisystem involvement in MD is still unclear, a role of oxidative stress in this disease has been suggested. We investigated 39 MD patients to assess the plasma concentration of advanced oxidation protein products (AOPPs) and ?-glutamyltransferase (GGT) and related them to clinical severity scores. Plasma AOPP levels (p=0.021), total serum GGT activity (p=0.0005) and GGT activity associated with low-density lipoprotein (p=0.0021) were significantly higher in patients than in controls. There was significant correlation between serum GGT levels and AOPPs (r=0.5831; p=0.0022). A statistically significant increase in serum GGT with age was found in MD patients (p=0.0193). Receiver operating characteristic curve analysis showed that higher AOPP levels were significantly associated with extra-muscular signs of the disease, i.e., cataracts and heart involvement (area under the curve±SE=0.908±0.083), but not with muscular involvement. The concomitant increment in GGT and AOPPs indicates a possible role of oxidative stress in the pathogenesis of MD type 1, while the association of increased AOPP levels with extra-muscular signs of the disease suggests that individual susceptibility to oxidative stress can modulate the extra-muscular phenotype of the disease.
Clinical Chemical Laboratory Medicine, Walter de Gruyter
Print ISSN: 1434-6621
Volume: 43, 07/2005
Pages: 745 - 747