Espeel M, Jauniaux E, Hashimoto T, Roels F
Free University Brussels, Laboratory of Human Anatomy, Belgium.
Prenat Diagn. 1990 Jun;10(6):349-57. doi: 10.1002/pd.1970100602.
In the majority of congenital peroxisomal disorders, beta-oxidation of very long chain fatty acids is deficient. We have investigated the appearance and localization of the three peroxisomal beta-oxidation enzymes in normal fetal liver (fertilization age between 5 and 18 weeks) with protein A-gold immunocytochemistry and silver enhancement for light microscopic visualization. With specificity-tested polyclonal antibodies, acyl-CoA-oxidase, bifunctional enzyme, and 3-oxoacyl-CoA thiolase were localized in the peroxisomes of the parenchymal cells, which appear as brown or black granules. In the youngest specimen, no immunopositive reaction was obtained. A weak reaction with anti-thiolase was obtained at the age of 6-7 weeks. At a fertilization age of 8 weeks, peroxisomes could be distinctly visualized after immunostaining for all three enzymes. From a staining series with anti-thiolase on simultaneously treated slides, it appears that the amount of antigen per peroxisome and the organelle size increase between the seventh and eighteenth weeks. These data should enable a more specific diagnosis in fetal liver biopsies from pregnancies at risk and after termination of pregnancy.
在大多数先天性过氧化物酶体疾病中，极长链脂肪酸的β-氧化存在缺陷。我们运用蛋白A-金免疫细胞化学和银增强技术进行光镜观察，研究了正常胎儿肝脏（受精年龄在5至18周之间）中三种过氧化物酶体β-氧化酶的出现情况和定位。使用经过特异性测试的多克隆抗体，酰基辅酶A氧化酶、双功能酶和3-氧代酰基辅酶A硫解酶定位于实质细胞的过氧化物酶体中，呈现为棕色或黑色颗粒。在最年幼的标本中，未获得免疫阳性反应。在6至7周龄时，与抗硫解酶抗体出现微弱反应。在受精年龄为8周时，对所有三种酶进行免疫染色后，过氧化物酶体可清晰显现。从同时处理的载玻片上用抗硫解酶进行的一系列染色结果来看，在第7周至第18周期间，每个过氧化物酶体的抗原量和细胞器大小都有所增加。这些数据应有助于对有风险妊娠及终止妊娠后的胎儿肝脏活检进行更准确的诊断。
