RT期刊文章SR电子T1辅酶Q10水平下降在多个系统小脑萎缩(P4.304)摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP P4.304 VO 86是16补充A1 Emanuele巴萨A1朱利首页奥Kleiner A1 Goumei唐A1萨特林A1 Chi-Ying A1以利以谢Masliah A1 Sheng-Han郭A1卡塔琳娜州Quinzii Hirano年2016 UL //www.ez-admanager.com/content/86/16_Supplement/P4.304.abstract AB目的:辅酶Q10缺乏最近发表在家族和零星的多系统萎缩(MSA)的患者,与COQ2基因的突变有关。为了调查是否缺辅酶q代表了一种普遍现象在MSA的大脑,独立于COQ2突变。方法:我们进行了一项病例对照研究比较辅酶q10水平的线粒体功能12多个系统的后期大脑萎缩的情况下,12个年龄组和帕金森病的情况下,特别是:COQ2序列,辅酶q10水平,线粒体呼吸链酶活性、氧化应激、线粒体质量,和蛋白质水平的酶参与公鸡生物合成和/或其监管评估。结果:直接序列COQ2任何疾病变异MSA的大脑是负面的。辅酶q10水平显著下降相比,MSA小脑控制小脑(68.1±34.7 nmol辅酶q /毫克蛋白和113.1±24.8 nmol辅酶q /毫克蛋白,p = 0.001),未发现差异和MSA和帕金森病和cerebellem之间。在MSA样本,呼吸链酶活性是正常的。特殊染色法对氧化膜脂质透露的一个子集4-hydroxynonenal浦肯野细胞增加的信号,表明广泛的氧化应激。我们所做的。我们评估了公鸡生物合成途径,测量稳态水平的蛋白质参与公鸡合成及其调控。蛋白质水平的PDSS1 (COQ1)和COQ5在MSA的大脑相比,显著降低控制大脑。 Conclusions: Our findings showed decreased CoQ10 levels, associated with impaired CoQ biosynthesis, and oxidative stress in multiple system atrophy cerebellum, in the absence of COQ2 mutations, thus suggesting that CoQ10 deficiency could represent a common, secondary phenomenon in MSA. This study points out that CoQ10 might play a role in the pathogenesis of MSA and, since no disease modifying therapies are available, interventions on the CoQ10 biosynthetic pathways might be a compelling strategy to treat MSA.Disclosure: Dr. Barca has nothing to disclose. Dr. Kleiner has nothing to disclose. Dr. tang has nothing to disclose. Dr. Tadesse has nothing to disclose. Dr. Lin has nothing to disclose. Dr. Masliah has nothing to disclose. Dr. Kuo has nothing to disclose. Dr. Quinzii Hirano has nothing to disclose.Tuesday, April 19 2016, 8:30 am-7:00 pm