首页> 美国卫生研究院文献>Orphanet Journal of Rare Diseases >Stilbenes and resveratrol metabolites improve mitochondrial fatty acid oxidation defects in human fibroblasts
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Stilbenes and resveratrol metabolites improve mitochondrial fatty acid oxidation defects in human fibroblasts

机译:芪和白藜芦醇代谢物改善人成纤维细胞中的线粒体脂肪酸氧化缺陷

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摘要

BackgroundInborn enzyme defects of mitochondrial fatty acid beta-oxidation (FAO) form a large group of genetic disorders associated to variable clinical presentations ranging from life-threatening pediatric manifestations up to milder late onset phenotypes, including myopathy. Very few candidate drugs have been identified in this group of disorders. Resveratrol (RSV) is a natural polyphenol with anti-oxidant and anti-inflammatory effects, recently shown to have beneficial metabolic properties in mice models. Our study explores its possible effects on FAO and mitochondrial energy metabolism in human cells, which are still very little documented.
机译:背景线粒体脂肪酸β-氧化(FAO)的先天性酶缺陷形成了大量遗传疾病,与各种临床表现相关,从威胁生命的儿科表现到较轻的晚发型,包括肌病。在这组疾病中几乎没有发现候选药物。白藜芦醇(RSV)是具有抗氧化和抗炎作用的天然多酚,最近在小鼠模型中显示具有有益的代谢特性。我们的研究探索了其可能对人类细胞中FAO和线粒体能量代谢的可能影响,目前尚无文献报道。

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