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An impairment of mitochondrial function may contribute to the pathophysiology of myotonic dystrophy (MyD). Coenzyme Q10 (CoQ10) deficiency has been previously observed, even if in a restricted sample of patients. The aim of this investigation was to obtain more information about coenzyme Q10 and its relationships to the aerobic metabolism in a group of MyD patients. Serum CoQ10 appeared significantly reduced with respect to normal controls: 0.93 +/- 0.22 vs. 1.58 +/- 0.28 micrograms/ml (p < 0.05). Moreover, the results demonstrated an inverse tendency between CoQ10 levels and the CTG expansion degree. Basal blood lactate levels were significantly higher than controls (p < 0.05). A borderline inverse correlation between CoQ10 and lactate, corresponding to lactate threshold, was found. These data suggest a possible role of CoQ10 in the pathogenesis of MyD, which may be mediated by mechanisms of cellular damage common to the oxidative pathway. Therapeutic strategies may be devised by virtue of this rationale.

Trans, trans-muconic acid (tt-MA) is one of the most important metabolites of benzene, a pollutant ubiquitously distributed in ambient air and classified in 1982 as a group I carcinogen. For its sensitivity and specificity, tt-MA excreted in urine is considered a good biological marker of benzene exposure. In this study, seminal tt-MA levels in occupationally nonexposed subjects (n = 32) have been determined. The seminal fluid of normozoospermic subjects contained an average tt-MA concentration (170 +/- 100 ng ml-1) significantly lower than that of teratozoospermic (310 +/- 180 ng ml-1; P < 0.01), oligozoospermic (400 +/- 180 ng ml-1; P < 0.001), and oligoasthenozoospermic (430 +/- 230 ng ml-1; P < 0.01) subjects. A negative correlation existed between tt-MA levels and sperm concentration (r = - 0.62; P < 0.001), percentage of normal spermatozoa (r = - 0.41; P < 0.05), and percentage of vital spermatozoa (r = - 0.89; P < 0.001). Average tt-MA levels detected in seminal plasma were higher in smokers (350 +/- 160 ng ml-1) than in nonsmokers (280 +/- 210 ng ml-1). These results show that seminal plasma tt-MA content could be an important biological indicator for evaluating the negative effects of benzene on spermatogenesis.