EFFECT OF REYES-SYNDROME SERUM ON ISOLATED CHINCHILLA LIVER-MITOCHONDRIA
EFFECT OF REYES-SYNDROME SERUM ON ISOLATED CHINCHILLA LIVER-MITOCHONDRIA
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DOI：
10.1172/jci112039
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发表时间：
1985-01-01
影响因子：
15.9
通讯作者：
GETZ, GS
中科院分区：
文献类型：
--
作者：
TONSGARD, JH;GETZ, GS
肝脏线粒体酶的普遍损伤是雷氏综合征 (RS) 的核心。添加来自12名患者的浓缩正常血清或RS血清后，测量离体肝线粒体的呼吸。 RS 血清刺激离体大鼠肝线粒体中的 O2 消耗。这种效应是由于污染制剂的过氧化物酶体氧化尿酸以及刺激线粒体呼吸（1.05 .+-. 0.14 nmol O2/min .cntdot. mg 蛋白质；对照 0.30 .+-. 0.08 nmol O2/min .cntdot. mg）。呼吸刺激发生在所有呼吸底物存在的情况下，取决于添加的血清量，并且代表氧化磷酸化的解偶联。 RS 血清可减少 15-76% 的 ATP 形成。解偶联效应与血清样品中游离脂肪酸的量相关，并且类似于添加二羧酸脂肪酸引起的效应。二羧酸脂肪酸，尤其是长链二羧酸，会损害 ATP 的形成。在所有 RS 患者的血清中都发现了二羧酸，其含量高达血清游离脂肪酸总量的 54%。血清二羧酸中 90% 的长度为 16-18 C。 RS血清中二羧酸的量与RS血清中ATP形成的减少直接对应。二羧酸存在于 RS 中，并且可能在 RS 和其他存在二羧酸的疾病中线粒体功能的一般损害中发挥重要作用。
A general impairment of liver mitochondrial enzymes is central to Reye''s syndrome (RS). The respiration of isolated liver mitochondria was measured after the addition of concentrated normal serum or RS serum derived from 12 patients. RS serum stimulates O2 consumption in isolated rat liver mitochondria. This effect is due to the oxidation of uric acid by peroxisomes contaminating the preparation and a stimulation of mitochondrial respiration (1.05 .+-. 0.14 nmol of O2/min .cntdot. mg of protein; control 0.30 .+-. 0.08 nmol O2/min .cntdot. mg). The stimulation of respiration occurs in the presence of all respiratory substrates, is dependent on the amount of serum added and represents an uncoupling of oxidative phosphorylation. RS serum reduces ATP formation by 15-76%. The uncoupling effect correlates with the amount of free fatty acid in the serum sample and resembles the effect induced by the addition of a dicarboxylic fatty acid. Dicarboxylic fatty acids, especially long-chain dicarboxylic acids, impair ATP formation. Dicarboxylic acids were found in the serum of all RS patients and comprised as much as 54% of the total serum free fatty acids. Of the serum dicarboxylic acids 90% were of 16-18 C lengths. The amount of dicarboxylic acids in the RS serum corresponded directly with the reduction in ATP formation by the RS serum. Dicarboxylic acids occur in RS and may be important in the general impairment of mitochondrial function in RS and other disorders where they are present.