ArticleVirchows Archiv. A, Pathological anatomy and histopathology1991
Peroxisomal localization of the immunoreactive beta-oxidation enzymes in a neonate with a beta-oxidation defect. Pathological observations in liver, adrenal cortex and kidney.
Article in Virchows Archiv. A, Pathological anatomy and histopathology, 1991. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- A brief history of the human liver peroxisome.Histochemistry and cell biology · 2026Review
- In situ heterogeneity of peroxisomal oxidase activities: an update.The Histochemical journal · 1996Review
- Oxidation of pristanic acid in fibroblasts and its application to the diagnosis of peroxisomal beta-oxidation defects.The Journal of clinical investigation · 1996Article
- Immunocytochemical localization of peroxisomal proteins in human liver and kidney.Journal of inherited metabolic disease · 1995Article
- Secondary alterations of human hepatocellular peroxisomes.Journal of inherited metabolic disease · 1995Review
- Measurement of dihydroxyacetone-phosphate acyltransferase (DHAPAT) in chorionic villous samples, blood cells and cultured cells.Journal of inherited metabolic disease · 1995Article
- Practical guide for morphometry of human peroxisomes on electron micrographs.Journal of inherited metabolic disease · 1995Review
- Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts.Journal of inherited metabolic disease · 1995Article
- Immunoblot analysis of peroxisomal proteins in liver and fibroblasts from patients.Journal of inherited metabolic disease · 1995Article
- Liver and chorion cytochemistry.Journal of inherited metabolic disease · 1995Article
- Liver pathology and immunocytochemistry in congenital peroxisomal diseases: a review.Journal of inherited metabolic disease · 1991Review
- Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency): novel data.Virchows Archiv. A, Pathological anatomy and histopathology · 1991Article
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7 authors.
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Abstract
A boy born to healthy, unrelated parents, presented at birth with hypotonia and seizures. Very long chain fatty acids in the plasma were strongly elevated; bile acid intermediates and plasmalogen biosynthesis were normal. Acyl-CoA oxidase activity was normal. The patient died at the age of 3 months. The cerebellum and medulla oblongata showed neuronal migration defects. The specific biochemical basis for the impaired peroxisomal beta-oxidation has not been found. The three immunoreactive peroxisomal beta-oxidation enzymes and catalase were localized in the hepatocellular peroxisomes. Aberrant features of the peroxisomes included: a subpopulation of organelles larger than 1 micron, an amorphous nucleoid in many organelles, and invaginations of the peroxisomal membrane into the matrix. Peroxisomes in the proximal renal tubules also contained the three immunoreactive beta-oxidation enzymes. Regularly spaced trilamellar inclusions were seen in hepatic macrophages; they were much more abundant in adrenocortical macrophages. The inclusions were birefringent and resistant to acetone extraction. Distinct hepatic fibrosis had developed over a period of 2.5 months. We speculate that the impaired beta-oxidation is due to a defect at the level of the peroxisomal carnitine octanoyl or -acetyl transferase, responsible for the export of beta-oxidation products.
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