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首页> 外文期刊>Neurochemistry International: The International Journal for the Rapid Publication of Critical Reviews, Preliminary and Original Research Communications in Neurochemistry >Free radical release in C6 glial cells enriched in hexacosanoic acid: implication for X-linked adrenoleukodystrophy pathogenesis.
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Free radical release in C6 glial cells enriched in hexacosanoic acid: implication for X-linked adrenoleukodystrophy pathogenesis.

机译：富含六烷酸的C6神经胶质细胞中的自由基释放：对X联肾上腺白质营养不良症发病机制的影响。

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摘要

Free radicals have been implicated in the etiopathology of some neurological and demyelinating diseases. To evaluate their involvement in the cerebral form of X-linked adrenoleukodystrophy (cerALD) disorder, characterised by very long chain fatty acid (VLCFA) accumulation, we utilised an in vitro model using rat C6 glial cells, enriched in hexacosenoic acid (C26:0, HA). Modified cells were incubated in presence of oxidative stressors, such as bacterial endotoxin lipopolisaccharides (LPS) and human oxidised low-density lipoprotein (ox-LDL), and the production of proinflammatory cytokines, nitrite, nitrate and superoxide was determined in the supernatants. The results show that modified cells produce higher amounts of nitric oxide (NO) products and superoxide compared to native C6 cells, supporting the role of free radicals as important pathophysiological modulator of the neuroinflammatory response in ALD. This hypothesis suggests that the cerebral damage in ALD could be due to intracellular signalling activated by interaction of exogenous factors with the particular membrane fatty acid composition.
机译：自由基已经牵涉到一些神经系统疾病和脱髓鞘疾病的病因病理中。为了评估他们参与以超长链脂肪酸（VLCFA）积累为特征的X联肾上腺髓质营养不良（cerALD）疾病的脑型，我们利用了大鼠C6神经胶质细胞的体外模型，该细胞富含六烯酸（C26：0 ， 哈）。将修饰的细胞在氧化应激源（例如细菌内毒素脂寡糖（LPS）和人氧化的低密度脂蛋白（ox-LDL））存在下孵育，并在上清液中确定促炎性细胞因子，亚硝酸盐，硝酸盐和超氧化物的产生。结果表明，与天然C6细胞相比，修饰过的细胞产生更多的一氧化氮（NO）产物和超氧化物，支持自由基作为ALD神经炎症反应的重要病理生理调节剂的作用。该假设表明，ALD中的脑损伤可能是由于外源因子与特定膜脂肪酸成分相互作用而激活的细胞内信号传导所致。

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