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- W3030893377 abstract "目的 研究硒蛋白-P与琥珀酸脱氢酶、细胞色素C氧化酶的关系及它们在线粒体疾病患者不整红边纤维(ragged redfiber,RRF)中的表现和作用.方法(1)研究对象:线粒体疾病患者14例,其中Kearns-Sayre综合征患者1例,线粒体脑肌病、乳酸酸中毒伴卒中样症状者4例,肌阵挛癫癎伴RRF 1例,慢性进行性眼外肌麻痹6例,肢带型线粒体病2例;5例对照,其中多发性肌炎2例,其他神经肌病3例.全部患者均经临床、病理、分子生物学DNA分析确诊.(2)开放式肌肉活检.(3)连续切片,经组织化学、免疫组织化学染色分析.结果患者RRF的肌浆中硒蛋白-P显著表达,琥珀酸脱氢酶活性增强,细胞色素C氧化酶活性增强、减弱或缺陷.结论硒蛋白-P在对抗线粒体氧化损伤、保护线粒体机能方面可能充当了重要角色,可以作为病理学评价线粒体机能和诊断RRF的指标。" @default.
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- W3030893377 date "2005-05-23" @default.
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- W3030893377 title "The studies of characteristic expression and effect of selenoprotein P in ragged red fibers in patients with mitochondria disorders" @default.
- W3030893377 doi "https://doi.org/10.3760/j.issn:1006-7876.2005.05.011" @default.
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