Epigallocatechin-3-Gallate Plus Omega-3 Restores the Mitochondrial Complex I and F(0)F(1)-ATP Synthase Activities in PBMCs of Young Children with Down Syndrome: A Pilot Study of Safety and Efficacy.
Epigallocatechin-3-Gallate Plus Omega-3 Restores the Mitochondrial Complex I and F(0)F(1)-ATP Synthase Activities in PBMCs of Young Children with Down Syndrome: A Pilot Study of Safety and Efficacy.
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Epigallocatechin-3-Gallate Plus Omega-3恢复了线粒体复合物I和F（0）F（0）F（1）-ATP合酶活性在患有唐氏综合症的幼儿的PBMC中：安全性和有效性的试点研究。
DOI：
10.3390/antiox10030469
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发表时间：
2021-03-16
期刊：
影响因子：
--
通讯作者：
Strisciuglio P
中科院分区：
文献类型：
--
作者：
Scala I;Valenti D;Scotto D'Aniello V;Marino M;Riccio MP;Bravaccio C;Vacca RA;Strisciuglio P
唐氏综合征（DS）是智力残疾的主要遗传原因。DS的发病机制尚未完全阐明，也没有特定的药物治疗。DYRK 1A过表达，氧化应激和线粒体功能障碍在21三体中被描述。表没食子儿茶素-3-没食子酸酯（EGCG）是一种具有抗氧化特性的多模式营养品。EGCG抑制DYRK 1A过表达并在体外纠正DS线粒体功能障碍。本研究探讨了接受EGCG治疗的1-8岁DS儿童的安全性特征（10 mg/kg/die，在空腹条件下，口服omega-3混悬液，持续6个月），以及EGCG在恢复线粒体复合物I和F0 F1-ATP合酶（复合物V）缺陷方面的疗效，对PBMC进行了评估。采用Griffiths精神发育量表-扩展修订版（GMDS-ER）进行发育分析。结果表明，脱咖啡因的EGCG（>90%）加omega-3在DS儿童中是安全的，并有效地恢复线粒体复合物I和V活性的缺陷。在21%的接受EGCG治疗的患者中观察到血浆叶酸水平下降，应仔细监测。与DS对照组相比，给药组的GMDS-ER评分未显示差异。总之，在1-8岁的DS儿童中，可以在医疗监督下安全地给予EGCG加omega-3，以使线粒体呼吸链复合体活动正常化，而对发育性能改善的结果仍不确定。
Down syndrome (DS) is a major genetic cause of intellectual disability. DS pathogenesis has not been fully elucidated, and no specific pharmacological therapy is available. DYRK1A overexpression, oxidative stress and mitochondrial dysfunction were described in trisomy 21. Epigallocatechin-3-gallate (EGCG) is a multimodal nutraceutical with antioxidant properties. EGCG inhibits DYRK1A overexpression and corrects DS mitochondrial dysfunction in vitro. The present study explores safety profiles in DS children aged 1–8 years treated with EGCG (10 mg/kg/die, suspended in omega-3, per os, in fasting conditions, for 6 months) and EGCG efficacy in restoring mitochondrial complex I and F0F1-ATP synthase (complex V) deficiency, assessed on PBMCs. The Griffiths Mental Developmental Scales—Extended Revised (GMDS-ER) was used for developmental profiling. Results show that decaffeinated EGCG (>90%) plus omega-3 is safe in DS children and effective in reverting the deficit of mitochondrial complex I and V activities. Decline of plasma folates was observed in 21% of EGCG-treated patients and should be carefully monitored. GMDS-ER scores did not show differences between the treated group compared to the DS control group. In conclusion, EGCG plus omega-3 can be safely administered under medical supervision in DS children aged 1–8 years to normalize mitochondria respiratory chain complex activities, while results on the improvement of developmental performance are still inconclusive.
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影响因子：
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作者：
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