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首页> 外文期刊>Life sciences >Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain.
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Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain.

机译：大鼠脑中LCHAD和MTP缺乏症中积累的代谢物引起的线粒体能量稳态紊乱。

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摘要

AIMS: We investigated the in vitro effects of 3-hydroxydodecanoic (3HDA), 3-hydroxytetradecanoic (3HTA) and 3-hydroxypalmitic (3HPA) acids, which accumulate in tissues of patients affected by mitochondrial trifunctional protein (MTP) and isolated long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiencies, on various parameters of energy homeostasis in mitochondrial preparations from brain of young rats. MAIN METHODS: We measured the respiratory parameters state 4, state 3, respiratory control ratio (RCR) and ADP/O ratio by the rate of oxygen consumption, as well as the mitochondrial membrane potential and the matrix NAD(P)H levels in the presence of the fatty acids. KEY FINDINGS: We found that 3HDA, 3HTA and 3HPA markedly increased state 4 respiration and diminished the RCR using glutamate plus malate or succinate as substrates. 3HTA and 3HPA also diminished the mitochondrial membrane potential and the matrix NAD(P)H levels. In addition, 3HTA decreased state 3 respiration using glutamate/malate, but not pyruvate/malate or succinate as substrates. Our data indicate that the long-chain 3-hydroxy fatty acids that accumulate in LCHAD/MTP deficiencies act as uncouplers of oxidative phosphorylation, while 3HTA also behaves as a metabolic inhibitor. SIGNIFICANCE: It is presumed that impairment of brain energy homeostasis caused by these endogenous accumulating compounds may contribute at least in part to the neuropathology of LCHAD/MTP deficiencies.
机译：目的：我们研究了3-羟基十二烷酸（3HDA），3-羟基十四烷酸（3HTA）和3-羟基棕榈酸（3HPA）酸的体外作用，这些酸积累在受线粒体三功能蛋白（MTP）和分离的长链影响的患者的组织中3-羟酰基-CoA脱氢酶（LCHAD）缺陷，来自幼鼠脑线粒体制剂中能量稳态的各种参数。主要方法：我们测量了呼吸参数状态4，状态3，呼吸控制率（RCR）和ADP / O率，方法是耗氧率，线粒体膜电位和基质中NAD（P）H的水平。脂肪酸的存在。主要发现：我们发现，以谷氨酸加苹果酸或琥珀酸为底物，3HDA，3HTA和3HPA显着提高了4状态呼吸，并降低了RCR。 3HTA和3HPA还减少了线粒体膜电位和基质NAD（P）H水平。另外，3HTA使用谷氨酸/苹果酸降低了状态3的呼吸，但没有丙酮酸/苹果酸或琥珀酸酯作为底物。我们的数据表明，在LCHAD / MTP缺陷中积累的长链3-羟基脂肪酸可作为氧化磷酸化的解偶联剂，而3HTA也可作为代谢抑制剂。意义：据推测，由这些内源性积累化合物引起的脑能量稳态障碍可能至少部分地导致了LCHAD / MTP缺陷的神经病理学。

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