Miles Lili, Miles Michael V, Tang Peter H, Horn Paul S, Quinlan John G, Wong Brenda, Wenisch Alexandra, Bove Kevin E
Division of Pathology and Laboratory Medicine, Department of Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, United States.
Clin Chim Acta. 2005 Oct;360(1-2):87-96. doi: 10.1016/j.cccn.2005.04.009.
Coenzyme Q (CoQ) has been suggested as a biomarker for tissue redox status. The aims are (1) to compare ubiquinol-9, ubiquinol-10, ubiquinone-9, ubiquinone-10, total CoQ content and CoQ redox ratio in quadriceps muscle, heart, brain and liver tissues of mdx mice with wild-type controls; and (2) to determine if ubiquinol content and CoQ redox ratio changes are associated with pathological findings in mdx mouse.
CoQ contents were determined in homogenized quadriceps muscle, heart, liver and brain of age-matched mdx and wild-type control mice by HPLC-EC. Light and electron microscopy studies were conducted using standard pathology methods.
Ubiquinol-9 and ubiquinol-10 concentrations are significantly increased in quadriceps and heart muscle of mdx mouse. Increased redox ratios of coenzyme Q(9) and coenzyme Q(10) are also evident in quadriceps, heart and liver tissues in mdx mouse, but not brain. Pathological examination shows marked myofiber regeneration and evidence of mitochondrial proliferation for mdx muscle.
Evidence that changes in ubiquinol content and CoQ redox ratio are related to pathological features in mdx skeletal and heart myofibers suggests that tissue ubiquinol content and CoQ redox ratio may be useful biomarkers for evaluating muscle disorders associated with mitochondrial proliferation and defects in oxidative phosphorylation.
辅酶Q(CoQ)已被认为是组织氧化还原状态的生物标志物。目的是:(1)比较mdx小鼠与野生型对照小鼠的股四头肌、心脏、脑和肝组织中泛醇-9、泛醇-10、泛醌-9、泛醌-10、总CoQ含量及CoQ氧化还原比;(2)确定泛醇含量和CoQ氧化还原比的变化是否与mdx小鼠的病理表现相关。
采用高效液相色谱-电化学检测法(HPLC-EC)测定年龄匹配的mdx小鼠和野生型对照小鼠股四头肌、心脏、肝脏和脑匀浆中的CoQ含量。使用标准病理学方法进行光镜和电镜研究。
mdx小鼠股四头肌和心肌中泛醇-9和泛醇-10浓度显著升高。mdx小鼠的股四头肌、心脏和肝脏组织中辅酶Q(9)和辅酶Q(10)的氧化还原比也明显升高,但脑内未升高。病理检查显示mdx肌肉有明显的肌纤维再生和线粒体增殖迹象。
泛醇含量和CoQ氧化还原比的变化与mdx骨骼肌和心肌纤维病理特征相关的证据表明,组织泛醇含量和CoQ氧化还原比可能是评估与线粒体增殖和氧化磷酸化缺陷相关的肌肉疾病的有用生物标志物。