4-Hydroxynonenal: a specific indicator for canine neuronal-retinal ceroidosis.
4-Hydroxynonenal: a specific indicator for canine neuronal-retinal ceroidosis.
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4-羟基壬烯醛：犬神经元-视网膜蜡样硬化症的特异性指标。
DOI：
10.1002/ajmg.1320310620
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
Koppang,N
中科院分区：
文献类型：
--
作者：
Siakotos,AN;Bray,R;Dratz,E;vanKuijk,F;Sevanian,A;Koppang,N
先前试图证明各种形式的神经元蜡样脂褐质沉积症（NCL）中脂质过氧化异常的尝试一直没有成功，包括过氧化物水平（过氧化物酶）。在本实验中，为了研究不饱和脂肪酸急性氧化产物4-羟基壬烯醛（HNE）的相对浓度，对NCL犬模型和正常对照犬的外周血细胞和各种组织进行了检测。HNE与O-（2，3，4，5，6-五氟苄基）羟胺反应形成4-羟基壬烯醛（O-五氟苄基）肟后进行测定。该反应产物用毛细管气相液相色谱法（g/c）分离，用火焰离子化法定量，结果表明，与正常对照动物相比，从患犬和携带者中分离的中性粒细胞中含有异常量的HNE。两名携带者的平均值高于正常值+3，289%，两只受影响动物的中性粒细胞高于正常值+4，873%。此外，与对照动物相比，受影响的狗的脑、视网膜、视网膜色素上皮（RPE）和肾中的相对HNE水平的检查也显示出HNE的异常水平，特别是在脑（+168%）和RPE（+135%）中，这两个器官表现出这些疾病特有的最严重的病理损伤。明确记录了HNE在这种犬形式的人NCL中的作用。HNE和其他α，β不饱和醛的众所周知的细胞毒性特性表明在这种疾病的发病事件中起主要作用。
Previous attempts to demonstrate abnormalities in lipid peroxidation in various forms of the neuronal ceroid‐lipofuscinoses (NCL) have been unrewarding up to and including the peroxide level (peroxidase). In this experiment a survey was made in a canine model of NCL to study the relative concentration of 4‐hydroxynonenal (HNE), a fragment derived from an acute oxidation product of unsaturated fatty acids.Peripheral blood cells and various tissues from an affected and a normal control dog were surveyed. HNE was assayed after reacting with O‐(2,3,4,5,6‐pentafluorobenzyl) hydroxylamine to form the 4‐hydroxynonenal (O‐pentafluorobenzyl) oxime. This reaction product was then separated by capillary gas liquid chromatography (g/c) and quantitated by flame ionization.The survey showed that neutrophils isolated from affected dogs and carries contained abnormal amounts of HNE when compared with normal control animals. Two carriers had mean values of +3,289% above normal, and neutrophils from two affected animals were +4,873% above normal. In addition, an examination of the relative HNE levels in brain, retina, retinal pigment epithelium (RPE), and kidney of an affected dog compared with a control animal also showed abnormal levels of HNE, particularly in brain (+168%) and in RPE (+135%), the two organs exhibiting the most severe pathologic damage unique to these disorders.These findings, although preliminary, clearly document a role for HNE in this canine form of human NCLs. The well‐known cytotoxic properties of HNE and other α,β unsaturated aldehydes suggest a primary role in the pathogenetic events of this disorder.