与青春期大鼠肝脏中 cis-5-十四碳烯酸和肉豆蔻酸诱导的通透性转换孔开放相关的线粒体功能紊乱

Disturbance of mitochondrial functions associated with permeability transition pore

opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats

Keywords：Cis-5-tetradecenoic acid, Liver mitochondria, Mitochondrial homeostasis, Mitochondrial permeability transition, Myristic acid, VLCAD deficiency 

关键词：Cis-5-十四碳烯酸、肝线粒体、线粒体稳态、线粒体通透性转变、肉豆蔻酸、VLCAD 缺陷

哺乳动物：大鼠

组织：肝脏

细胞系：其他细胞系

作者：Cecatto C、Amaral AU、Wajner A、Wajner SM、Castilho RF、Wajner M
出版期刊：《Mitochondrion》 （2020）

Abstract：

Patients affected by very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency commonly present liver dysfunction whose pathogenesis is poorly known. We demonstrate here that major metabolites accumulating in this disorder, namely cis-5-tetradecenoic acid (Cis-5) and myristic acid (Myr), markedly impair mitochondrial respiration, decreasing ATP production in liver mitochondrial preparations from adolescent rats. Other parameters of mitochondrial homeostasis such as membrane potential (ΔΨm) and Ca2+retention capacity were strongly compromised by these fatty acids, involving induction of mitochondrial permeability transition. The present data indicate that disruption of mitochondrial bioenergetics and Ca2+homeostasis may contribute to the liver dysfunction of VLCAD deficient patients.

文章摘要：

受极长链酰基辅酶A脱氢酶（VLCAD）缺乏症影响的患者通常会出现肝功能障碍，其发病机制知之甚少。 我们在此证明，在这种疾病中积累的主要代谢物，即 cis-5-十四碳烯酸 (Cis-5) 和肉豆蔻酸 (Myr)，会显着损害线粒体呼吸，降低青春期大鼠肝脏线粒体制剂中 ATP 的产生。 线粒体稳态的其他参数，如膜电位 (ΔΨm) 和 Ca2+ 保留能力受到这些脂肪酸的强烈影响，包括线粒体通透性转变的诱导。 目前的数据表明，线粒体生物能量学和 Ca2+ 稳态的破坏可能导致 VLCAD 缺陷患者的肝功能障碍。

点击链接即可查看和下载文章：https://linkinghub.elsevier.com/retrieve/pii/S1567-7249(19)30121-7

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