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Immunocytochemical localization of peroxisomal β- oxidation enzymes in human fetal liver

By Marc F A M. Espeel, Eric Jauniaux, Takashi Hashimoto and Frank Roels


In the majority of congenital peroxisomal disorders, β-oxidation of very long chain fatty acids is deficient. We have investigated the appearance and localization of the three peroxisomal β-oxidation enzymes in normal fetal liver (fertilization age between 5 and 18 weeks) with protein Agold 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 reation 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.SCOPUS: ar.jFLWNAinfo:eu-repo/semantics/publishe

Topics: Gynécologie, Obstétrique, Génétique clinique
Publisher: 'Wiley'
Year: 1990
DOI identifier: 10.1002/pd.1970100602
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Provided by: DI-fusion
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